Dr. Robert Heiling Library, S.m.s.medical College, Jaipur
Kaposi's, Multiple Indiopathic Hemorrhagic Sarcoma
Front matter
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rbinder unbry 301
AITERICAN LECTURE SERIES
lupuu
The B.LVERSTONE DIVISION /
AXTERIC LA LECTURES IN DERMATOLOGY
Ekin by
ARTHUR C CURTIS, LLD
Clormar, Drparian DrTudory and Sido
YTYY of Jlxc V dne School
Trbor Hxlu
KAPOSI'S SARCOMA
Multiple Idiopathic
Hemorrhagic Sarcoma
By
SANIUEL M. BLUEFARB, B.S., MD, FACP
Amochota Profesor of Dermatoloa Northweden Untersty Medical School
Attending Dermatologia and Chairman, Department of Dermatology
Cook County Horrel
Attendi Dermatologia, Internt Administration Ranch Horped
Aiden Attending Star Chicrpo Way Homoril Hospital
Chican, mimob
CHARLES C TI O NAS
Springfield Ilino
PUBLISHER
U.S.A.
CHARLES C 1710X145 PUBLISHER
Ravonoy Hoor
301 327 Fax Laurence Arabe, Spangkal, unots, US
Publis Armst att Brow Connormal olhou by
HOME KETIM NOKTION LTA, OTT, ITELA
Wulandards na Cala by
T.
no Tao TO
This book protated by copyright lopot
dit may be reproduced i ny manner at
out niti pompa mtbe publis
Caprukt 1957
HARLES C T11031
PL BLISHER
Library of Coopew Cube Crd \umber 57 3594
Prothe Cristais lame
TO RICHARD
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INTRODUCTION In the dghty-t's years since hapoal first described Multiple Idiopathic Hemorrhagic Sarcoms, the disease has continuously stimulated futarest. In the court of time every conceivablo facet of the Interesting disease bas been focarded and dis cussed racial incidence, age and ser predisposition, terminal ogy and Dorology posible causative factor, pathogenesis, symptomatology hematologic aspects and treatments. There Is a result, e voluminou Hteraturo on the publect, so Large that to Leep abreast of it is almost impossible. Dr Bluefarb, to undertaking to review and to assess citically tids vust fund of accumulated knowledge, has rendered a trvaluablo service. Cathering the material for this monograph was obviously a laborious task. It is not traportant that he is unablo to ascolbo the disease to a single cause it is not important that his dis cussion of treatment cods on a depressing Dote. It is toportant that I ayatlable facts are bere recorded in one small mono graph. In this eadly acreoblo book for altlal malyuts of the various view u to the cause of the disease, a wober appraial of the varlow therapies that have been mggested, Inderd, a full consideration of every phase of the discurood ako, meny Hitle-known facts are beren recorded Dr Blocfarb is uniquely Atted for the task for he has long becne setion student of the cutaneous manifestations of hmphomas and of so-called reticulocodothelial disetre. Ho contributed dgnifiantly to the Hterature of this subject. He has presented the material in this monograph on Kaposl's carcoma dimply and in logical requetice It should long servo an authoritative source of reference Herbert Pattner VID
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Kapod's Sarcoma Chapter Pago Systeznic vascular disease 39 Derived from netve tissue 6. As a manifestation of Reticuloendothelial Syster Hyperplada 13 0 Other theories 18 B. dlatbud Anatomy 51 V Symptoms 70 A Subjective Symptoms 70 B. Objectivo Symptoons 72 1 Edema 9 Betonbago 78 3. Nodula 82 1 Ulcerution of nodules 5. Plaques and infiltrations 88 8. Unusual manifestations 89 7 Evolution of eruption 98 C. Hemogrun 88 Dlateraal Orgen Involvement 102 L Lymph nodes 100 2 Gastrointestinal tract involvement 108 3. Respdrutory tract 113 4 Splect Involyttant 114 3 Urogenital tract 115 6. Bone Involvement 116 7 Het involvement 119 & Raro involvement 192 E. Associated with Other Discesat Lympbamas 123 VL Diagnosis und Differential Dlagnosis 130
Contents
Page
136
Choper
VIL Progpodds
VUIL Trotinent
Radiation therapy
Ultraviolet Hghat therapy
Surgical therapy
Chenical therupy
Arsenical therapy
1410
140
143
143
1.15
References
lodet
187
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1 Broritz Leposu (1837 1902) A previoul: unpublbou pooto praph (through the courtesy of Paul E Becket, BID and Arthur C Coat's, 21.D) I HISTORICAL I. 1872 kapor (1302) first descrlbol e condition which bo named "Idiopathio Vultiplo Pigment Sarcoma of the skin." Tho present designation of Lapods Sarcoma w suggested by Korboer (1376) in 1801. Koposd agreed to this designetion, but tutoty years later (1325) changed it to "marcoma Idiopathicum multiplex hemathagkan, a natio ho believed wu moro accurately descriptive of the coodition, and woull ako lodicato tho souco of pigment (banonderin) ta tho lastonIn his classical description of the disease, Kapos noted the symmetry and multiplicity of tho ledons from tho onset os a characteristic of the disease. This report was based on Ave case, but be later added 90 cases which he had observed. He regarded the disease as distinctio and believed it to bo a special and peculiar form of round cell carcoma, which occasionally presented characteristic spindle cells.
II SYNONYMS TL VANITY of descriptivo terms suggested since Kapood s original report give evidence of the veried conceptions of this discare. There are differences regarding the histologło changes of the varlow lesions in one individual, as well us in portions of the camo lesion, which accounts, in part, for the various theories about this disease. car Writer 1889 Lepost 1872 Lapost 1978 Tantund 1853 Hardaway 1891 Babes 1650 Funk 1091 Loebner Synonym Primaren idiopathischen hauteur coma Sarcoma kllopathicum multiplex plg mcntorum Sarcome Idiopathictum telangiec todos Sercome cutis Anglosarcoma perithelialo fusocellulero Sarcomatosis gummatoides Sarcana idiopathicum multiplex hemorhagiau der extremiteden Sercoms kilopathicum multiplex hemorrhagicum Actosarcoma multiplex cutaneum telangiectodas Photties beworshagtsches RCTOSAT cold Sarcomata Idiopathtaa multiplida pigmentose cutis 1804 Kaposd 1891 Unna 1698 Tonnesoli 1890 Bemhardt 4 Synonyme 5 Year Idler 1699 Gilchrist 1899 Poepelow 1809 Seld Synonyms Anglosarcoma Acroangioma hemorrhagicum Granuloma multiplex heman bagi cum 1901 Radachi 1902 Lieberthal 1902 Pelagatti 1910 Sequere 1912 Rarch Angloerklobelioma cutanam Aludple idiopathlo bemorlaglo ser coma Arcona Crumplomd englomatodos Sarcoma cutaneum telAnglectaticum multiplex hapoals sarcoma Sarcoma bemangloeddotheliale intravasculino Gramuloma muluplaa hanathagi 1919 Sternberg 1913 Martinotti 1015 Sibley cun 1917 Geocher Sarcomatosto primitiva telangoctosica 1922 Bertaccini Angloendothelioma cutaneum 1825 Cuiffre Sarcomatosis telangiectata cutanca Idiopathica generalista 10.28 Gunnlot Vultiple anglosarcoma 1927 Hamli and Halil Perithchoma moltiplex Dodulorum avertor lymphangiectodes cu tantum 1928 Jludelo, Callilan Pseudosarcomatosis telapglectatica & Chede 1928 Pautrker und Pseudosarcoma (hapod) my oueuro- Diss vascular dysgeneris 19.23 Nicholas and Sarcomatose telargloctatique płg Favre m.cataire 1931 TramootADO & Hemangioendotheliomu cutaneum Fittipaldi 1032 Ilamdl & Rent Acoperitheliomu idiopathicum mul tipler CRICTDOm lymphanglec todos cutendum 6 Kapod's Sarcoma car Writer Synonyms 1934 Kuoncov Endoperithelioma sarcometoda multiplex idiopathicum (typus Kopost) 1835 Lang & Haslbofa Systematized anglomatosis 1938 Chasser & Angloreticuloendothelioma Remcy 1953 Ronchese and Angloreticulomatosis Karn ILI ETIOLOGY Incidence and Sea Και αν LAPOSLE LANCOM appears to be relatively common in cov topolitan Areas foch u New York City and Chicago u well un such countries as Italy Poland and Russia. In a review of the literature, Dorffel (50) found reports of 358 proved Cars and added 16 case. The total is undoubtedly higher then these Agures Indicato doce, in many largo citio, Laport tarcoma is Dot reported imless tho patient habits rural manifestations of the disease of the 358 cases reported to the literature, Dorffd found 323 pero men (Avo under 90 years of age) and 21 were women, all adults. Ho tabulated all conci reported up to 1951 in which the age was given, . total of 380 caser, which probably off on a fair representation of the age inchkence of Kapool's sarcoma Decado Catou Per Cerut Decodo Cara Per Cort Furst Second Third Fourth Fifth 5 10 21 01 1 2.8 3.9 & 9 16.1 Sluth Suvonth Eighth Viath 23.0 98.1 12.5 21 u 53 The locdeskce to highest between the ato of 50 and 70 yeanbelog ko frequent to young adults and rare in childrel It is known that this dixere usually affects older petion and to much more cocoman in meh) than in Wonn.co Honover Soudget persons are not immune Tho fint symptoms may 7 B kopos's Sarcoma appear early in life while the typical full sodromo occurs at a Latar dato, Of the 134 pidients reported up to 1839 Cboister and Romrcy (10) found only 5.9 per cent wero foden. From that date to 1950 we found it additional cases in which the ser was given, among which thero vene 15 women, or 96 pa cent of the total Therefore, it seems apparent that between 8 and 10 per cent of all cases reported occur in women. An instanco of the discuso affecting sercral manbas of a family was reported by Greco (85) His patient was a 63 year old men whose two brothen had died of the disease and a son of one of these brotherı bed fest developed the coditton. Tho patient had the classical picture of Lepois dar coms for Ave years. B. Occurrenco in the Young Lapost's sarcoma rarely affects cukuresh, only aght cares accurrlog m the first decade of life bure been recorded hoc sard (138) described an eight year old Chinese boy and Dorfd (58) reported a boy who dovelopel tho disease fol Loving trauma at the age of da year Vc Lean (157) patient was a fut and one-half year old child who presented a symmetrical, deep purplish discoloration of the face the cheeks were markedly edematous, hard and tense and this condition extended up to the lower lids and down to the lona Hp and chm. The lips were indorated and pigmented, and there iu a slight hemorrhage in the subcoafunctia of the left eye. The youngest patient was recorded by Charglo (39b) who stated that he had observed a six month oll infant with Kaposi's disease A petent having the anset at five years of age was presented at the Dermatological Conference of the Vliedsspp Valley And Central State Dermatological fuoco tion (37) This patient also had osseous changes in the left pubic bons, left illum and the humerus. Craham-Littlo (91) described a woman who had developed the disease at the age of eight ytan. Denzer and Leopold (31) reported a four Etolog 9 and one-hall old Amerta boy of Italian peredtage, wbo doveloped tvo hlus spots on his left leg which were attributed to trawna Thero were two purplish raised painless nodales, cach 2 cans in diameter on the middle third of the left leg and several dark firm purplish plaques on tho ankles His tologic esamination revealed Kapood's narcoma The lessons did not respond to roentgen my therapy and the patient died Avo months later The diagnosis was confirmed at autopsy herrel (133) desatbed a year okl Bantu boy who had no cutancow lesions and the diagnosis wu made on histologia amination of a lymph node and the spleen, Tho occurrenco of Kaposi's prouma in the second decado was reported by SeDear and Wien (232b) io a 15 year old girl Carson and Knowles (17) desalbed two caseh, ono a 17 year old boy and tho other a 15 year old girl Swear and Caro (322) reported a 18 year old Italian girl and Silver (239) patient developed the deserts at 14 year of age. The patient reported by Bochet (14) was 18 years old and tho case pre sented by Hedge (114) was an 18 year old boy C Geographical Distribution Tho widersprood impression that Kapoofs arcoma i bilted almost exclusively to Jews is pon beloved to be entoncoin. On tho bads of studies reported in the literature and personal observation, we do not believe that the race of the patient to en etiological factor in this discuse We are in agreement with Dorfid (59) who states that the distribution of the disease is geographical rather than racial, since the vast majority of patients appear to be Russian Polish or Northern Itahan. Wiso (2352) found that the cas recorded to Mew York at were almost all men of Italian or Galician birth. Among 50 patients from Northern Italy reported by De Amics (53) none wete Jewish in the literature Dorld (58) found 111 Italian, 50 Russian, 20 Polish, and + Jewish patients, most of whom were born in Anda or Poland but they werd not to cluded with cither the Rusdan or Polish groups lp the sam 10 Raposls Sarcoma mary Thero weto also 12 Austrians, 8 Amaicans, 7 Hungardens and 3 Germans, while the remainder of the 358 patients represented nearly overy nationellty in the cvilized world. Among patient described by Cholgar and Ramsey (10) they found 1 were Italian and 19 Jewish while 16 were from diverse localities and in 2 cucs tho loculty was not mentioned Tho disease appear to occur les fro quently in Germans, Scandinavians, English and Americans Hovever VcCarthy and Pack (153) found 30 (83 per ant) of the patients in their series wero Jows or Italiens, regardles of nativity where arranged on a goographical basis, Eastern Europo was represented by only 74 per cent, practically all of whom were Jewish or Itakan. Although the high incidenco of Jews and Italians in New York City should be considerod, they bolleve that these findings support tho theory of radial or biological proclivity In the series of 38 cases, VicCarthy and Pack (153) found eight patients (22 per cent) to be American bom Lanos (141) patient was a native American man whore family had realded here for two generations. In commenting on this aux Kingsbury (1316) stated that he had nover before observed this duceso in a native American Tbo patent described by Gillet (88) wus a 30 year old Alohammodan man and Gougerot, of al (92b) reported a 30 year old Armenian wonen. Lockerds (138) patient was a Chinese student who developed the diretso at dght years of age and Epsteins (65) czte was the first to be reported in a Japaneso wonen. first report fra India, occurring in e Hindų, was that of Banerjee (19) Kapool's sarcoma appears to bo comparatively raro in tho Negro rac Ells (64) patient was a ful-blooded Negro who was a native of the United States. He prezentod DuncOW pigmented, non-souly macules, up to 1 cm to diameter on the chest and obdomen, and many firmo, petnless cutaneow and sabcutaneow nodules of 3 mms to come lo stre, on the ums and legs Lowenthal (159) described a full bloocked Negro Enology il who had about a degen podalas on the foot and around the ankles Garners (86) patient, a 34 year old Negro had dixcreto and conftrent, deeply pigmented Dodules and plaques on all the catremities, elephantlade changes of the feet and deep purpkih liyidity of the hands A 50 yeur old American Negro, reported by Symmens (254) had a painful indurated area on the finger not tho result of Injury There were lover] bluish red nodules on the left hypothenar eminenco and Amilar autodoou podules at tho Hp of the littlo finger on the right hand as well as largo podulated plaquca on the thenar eminenco and in tho palin Thero was a seria of noduks along tho hypotheoir eminenco and a small plaque on tbo Hght wrist joint, fust above the pains, and tweaty or moro similar nodules, each about 0.5 cms. In dlometer an both enllo Joints Aegerter and Peale (2) roportod a 60 year old Negro who had attacks of paroxymal dyspace which often awakened him from sleep. His heart was moderutely enlarged to the left, and there was a blowing systolle murmur at tho apex, trummitted to tho axilla Tho Hver was enlarged. Ile died ruddenly and autopsy disclosed the partcardial no to be immensely dilated with over a liter of fresh, unclotted blood. The heart sbowed moderato concentric hypertrophy Arislog from the wall of the night atrfum just distal to, and to tho baso of the right auricle, wa Dodular hemorrhagic tumor muss, Dicaruring + cont at the bora Jout distel to, and to the right of this region, a somewhat smaller tumor arow from tho night arterial wall down to the anterior surface of both tho Hght and left dde of tho heart, and up over tho coows atarlons und, at the base of the norts, thTo WLTO Dumcrow plaquc of tumor tissue. However Syminen (31b) stated that bo heil Camined the histologlal sections of the cardiac tumon and dbagreed with the diagnosis of Erpost's sarcoma A 61 year old Negro who had developed small tumal on both feet aloe ycan pre fously was desaibed by Brunsthing (30) The lesions progressed to fm olve the entire solo und toe of the Hght foot and the Instep of the left foot. Tbero 12 hapost's Sarcoma wero numatus per dizod aod larger rouod, firm cutaneous nodules, pink to brownish tn color distributed over the wola and toes of both feet. A diagnosis of blastomycodi, proved histologically had been made soven years previously Thero fore Bransting belloved tho most plawiblo explanation would be that kaposi's arcoma developed on the background of an older mfectious process. A 13 year old Aegro with lessons in the oropharyny w described by Sytop and Krantz (253) Bluefarb and Webster (26c) reported . Negro who had Kaposi's sarcoma of the skin and lymphosarcoma of tho lymph noder. A fullblooded Negro wus reported by Perky and Llo 2 Lepous marcoma occurring to patient with hmphouercoma 3 Esposa sorcoma in Negro (198) and Hazed (113) stated that he had observed two Neyroes havtog Kaposfs sarcome The occurrence of poor's tercoma in Negroes has also been described by Pardo-Castello (190) and by Sneld (24) Smith and Senitz (213) described in 65 year old Aegro and Andrews (ta) presental an Ameri an mulatto with Leposi's sarcome. Findlay (72) believes that Kapods wrcoma is not purther Larh rare in West Africa, since there is an OccusTeD CO of at Etology 13 least one caso in 50,000 woog the young adult population In 1922, Joot and Laigret (128) described a Bantu Negro who developed multiple times of Kaposd's turcona. A native of the Belgten Coogo who had nodular lesions on tho upper extremities and motin was described by Rogowsky (209) who also mentioned that this the dah cure obcerved in this geographical are kerel (133) descobed a dx year old Bantu boy Tvro patlants, both Bentu men, were doctbod by Kaniner and Blurray (131) They found that 41 cases of Kapod's come affectiog Bento mal hed proviously been roported. This relatively high incide.co bo to striking contrast to the relatively few cases reported in Ameran Negroes and, therefore, thoy considered the porchility of a genetic factor D Predispostoy Factor There appears to be definito rolationship to curtain pro diaposing factors such « general a local rascular lestone, scleros, ectasias, beroorhaglc dlatheads, alema, trauma and alcohollan. A prodromal stage i marked by local cyanosis, cdema and alight Inflammation, or by definite vascular ectadeo According to Pack (188b) these patients axbibit a coum.com physical habitus, namely flat feet, short sturdy legs thick inkles, tight inelastic do md cocacbtent or antecedent ar culatory disturbances of the lower ontrunities, such as part cosities and edemua. The legs usually shop varicostos and nodular bleeding of the peripheral vessels, another indication of the vascular origin of this tumor Recent studies indicate lymphadema 4 a factor in the genel of angioblastic arcome, according to licCarthy and Pack (153) who state that lyophanglourcomu of the upper extremity developing fn surgical ekeplantlets are idential with Kapool's wrong. Stewart und Tieres (219) reported hmphanglosarcoma developing to tho edemnatus erms of women whose clephantiasis, following moestectomy had beco prexcot from slt to 4 years. They speculated on a common nisterol carcinogenic factor responsible not only for the englo
14
Kaposi's Soncora
7
Dr
1 Kupouus arcoma lo alegre.
5 Typical localization of Kapoor's premi
Ellolo 15 Lircoma of the arm, but abo for the patinary carcinoma which lal to radical mastectomy All as patient had cutaneous lexions which resembled Kapods arcowe both clinically and histologically Two smlar cases were reported by Aegitter od Pealo (9) and by Hensson (108) Both women developed Lapors sarcoma of the lower atremitles nine and 20 year, respectively after the case of massivo lympbedema which had resulted from infection Tho probooged lymphedkeur, lo both cases, appeared to constitute tho solo brown predispodng factor soce do other primary peoplasm had proviomly been detected 1 Occupation Approalmately 85 per cent of the reported casa uro of the laboring class and a scant 5 per cent aro white collar workers, eccorillog to Van Clevo and Hellwig (205) Sorcina (233) comroented on the fact that mort patlanta aro men of powerful build. The Zabodog class patients loclude those aposed to atremes of temperature as well as those who do manual Labor It has never been ad equately plafoed why office workers and professional persons Aro less frequently afected than Laborers, imless the element of trauma bi moro proco to occur in the latter group It is the opinion of JicCarthy and Ped (153) that tho Laboring occupation docs Do Decessarily predisposo to Lapodl's sarcome, but rather that the occupation requires the patient to be on his feet for long periods of time. This is furtha cropbodied when it is noted that most patients develop the Initial tumor on the feet or legs, and often have an abocated ceni. Trauna. Slechanical toode or infections injury oray also be a factor Emmples of mechanical Injury preceding the development of Lapod's parcona in the inſured ara aro frequently recorded Leberthal (1512) dexibed a man who had toured his leg and, one month later painful swelling and Dodules developed on the injured leg, which was the first smptom of the disesse. Ledermann (117) patient do veloped topical nodules on the ann falloutng borse bite 18 Kapod's Sarcoma three years provbowly New nodules oppoered in the area after a short Hmo. Ormsby and Alltchell (1882) reported. 58 year old Swedish man wbo developed the disease following gnet bites. Weber and Deset (973b) described a patient who doveloped nodules of the discuso efter stepplog op a ball. Hedgos (114) patient, an 18 year old boy had placed the right foot with a nall nine years proviously Tho man reportal by Sibley (237) hed aut his hand slightly on a tooth who dealing a blot eight years before. A small red spot appeared immediately afterward, which had gradually increased in doo and, 18 months later a pigmented lesion appeared on the bach of the hand. Later the feet and ankles beautio fnvolved and the condition gradually spread upward to the face, whilo the head and neck were the last areas to be imahed by tumon, Pick (1880) Wallhauser (288) Zumbusch (291) Dals Farera (52) Chargin (381) Jeansobno (120) and Fat (70) have also recorded cases in which there was little ques tion of the relationship between mechanical injury and tho subsequent appearance of tumor in the lojured arens and subsequently in the typical distribution of the disease 3. Cold. Exposure to cold has been mentioned u a factor in the development of nodules of Laposi's sarcoma. Semenow (231) observed 10 cases, within a five year period, and Dotcu that a largo proportion of these patients had been exposed to more or less severe and prolonged cold and he was inclined to attributo a cerwativo influence to this factor in ono of his coses the bluish podulos Occisonally disappeared spontaneowly but always reappeared in cold and damp woather Vaguo types of erytipelas immediately precerllog the appearance of podulos of Kaposi's sarcome, as well as lymphangitis and celluhtly, have been reported. 4. Vascular Changa. Arteriosclerosis and other vascular changes, such as those present in syphilis, have been reported frequently The vascular changes aro usually found at antopsy but their hugh Inddence is probably explicable on tho buds of the advanced age of the patients, accordiog to Dorffd (58) Etolo 17 In somo ases, do definito procoding factor was found, but often vagus, prodromal symptoms were procat, such urber Datic pains, which uro recurrent and subject to variations in weather us mentioned by Steiner (2-17) kod other. Pelagati (191 carol) decilbod a patient having paralysis of ono ddo of the body and it iu on thle perelyzed do that Kapood's sarcoma first developed. Tho coodition ahrayı remained more severo On this ddo whilo leo OVCTO manifestations WTO present on other areas of the body The patient desalbed by Uhlmann (962) bad fractured tho upper un with resulting paralytis. This extremity was first affected by Kaposi's sur COM 5. Aronia. A patlent who doveloped Kaposi's sarcoma apparcutly fobowing an crything from armphetamine, wu described by Lelgheb (148) A 78 year old man who had Kapod's sarcoma for 17 years was reported by Eldea (82) Ilo doveloped a cutaneous hom following intravenow infections of an arccalcol (Solarnon) This hom was removed and histological canination rovealed typical Kaposi's war COM E Sitos of Predilection Tho aldon is the most frequent site of prodilection in Lepost's sarcoma. The cutaneous surface of the lower and uppet tremita aro most frequently in dlved (75 to 85 per coot of tho Coss) although I area of the cutaneous surface o crempt The next most frequent dtes of Involvement are the submucose of tho couro gastrointestinal tract, the material geni talia, the superbedal and decp lytoph polas (aspecially tho retroperitoocal and mesenteric) Hyer and lungi. Les com mon ster of lovolvement are the tonsils, bones, larynx and conjunctivae. Uncommon sites include the spleen, pancreas Lidocys adrenak, testes, sploidy ml, trachen, bronchl and plur. Lesions aro attremely raro in the heart, central nervou sateru, peripheral nerves, boogie, bladet muscles, pituitary gland and brul while Imolittacat of the thyroid gland ory or uterus bas DOVET been reporta.
18 kapoof's Sarcoma 1 Extremitla. Lepost's uncome has a markod tadaxcy to Localizo on the extrezitles, and develops symmetrically with a typical errengement. The cutaneow lesions at first appear on the hands and feet, then on the forearms and logs, and Later include the arms and thighs. Primery cutendons sar comata, localized in this manne may be regarded as the Lepod type. (Frohlich: 83) In elever of Hanssons (108) patients, the first podulo appeared on the feet, ip di the first manifestation ww on the herals, and two had primary lestons on the lower legs. Pid (1882) also described the first localisation on the lower legs Stats (216) reported e caso in which the disease was limited to the hands Philippson (107) and Dalla Favera (52) con- 0 Blateul lesson of pows arcoma abonntog tendency toward tymmetry Ada the extremities us well u the aternal genitalia, common stos of the disease The eruption 1 wually unilateral at first, then becarna bata Lateral with a teulancy toward simmetry u tho disease progresses. Symmetrical lovplement of both lower atremita to designated stocking distribution. Omegally the at ditton i gcrtalized The lesions are most numerow at the Etiolog 19 palpbaal ends of an trenity and becomo spurst neer the trunk, which I rarely fnydhed until lato in the disease when the Internal organs are abo involved. Symmas (-a) states that whilo the distribution of tho cutaneous lesions are of Interest, their significance is not clear Ho found that lesions occur in greatest abundanco in the elda loumediately covering the bone and in lesser numbers to tho tn ova roft HRICS. Alost often the leslams first appear on the lower atrauties, not uncommonly they may litt eppar on the flogen, around the wrists and on the effected as tremities. The plateau-Hko formations wally attain their greatest size in tho immediato vicinity of the points, notably tho mall joints of the hands and feet. The cutaneous ladous appear to have a predilection for the less flmihlo slan ereus having a less plentful blood supply and appear to avoid tho moto canly movablo skin areas which have a more abundant rupply of blood. A 78 year old man, presented by Vlorso (174) first de veloped kesons on the toes and feet eight years previous to tho development of lesions on the dorsum of the handt Cougerot Add Burnler (22) described a 40 year oll man who had Dolular lesions, localbed only on the palms, of three monthus duration Conzales and Vidaurreta (91) reported a 5 year oll Iulian who first developed a mall tumor on tho index Roger Three months later mumcrow other nodules appeared on the dorsum of the hands, forearms, feet and lower legs Satte lelon were slightly ukerated, and others tended to forta confluent tumor. There was a generalized edema of the hands and fect and nodules to the lobe and pinna of tho Night car Costello (Sb) presented a patient with a soft compressible telangiectatic, marble-sized lesion on tho right palme which was thought to be a granuloma bogenkuom. Zainon (9802 ) described a 3 year old man who had developed dushi and sharply outlined iniltrated plaques on the dorsum of the right hand smo mi prostouh FORD: (Ha) patient Husa 58 car old man wbose only kartons wete an eruption on the small finger of the left hand Kapast's Serconia which slowly extended to form a patch of purplish In Altration, fairly sharply defined, on the back of the hand, and smilar ureu on the fingert. A 21 year old man, reported by Formen (79b) had the initial lesions on tho ulnar border of the Hight hand while, et tho same time, lesions occurred on the left heel Roxburgh and Kleber (215) presented a 11 year old man wbo developed coalescent groups of raised, firm, smooth, dis 5 hello 7 Nodulo on tho too u the first manifestation of Laposi's sarcoa. 8 Modular onglomatow lexon of tho um resonblbog granatown pyogenicum cocte, purplo Dodulos, covered by a thin loose sale on tho Hght middle and immediately prodlmal to this too. The Dall was grooved and largely occluded by two superimposed Anglokctatonis, artslog from the superfidal aspect of tho nail fold. All of the cases orginally described by Kaposl, and most of those observed since, have exhibited some degree of yomotry Klaber (135c) descubed a 32 year old women in wbom the disease had remained unilateral tho year after onset The 3 year old Polish man, described by Kingsbury (1312) Etolog 21 doveloped the croption on the left inde finger ono od onohalf your previously and on the right foot afne ponths later Tho sto of the afected treu wu papllah to color with numerous hemorrhagic podulos. Rettner and Noubansa (201) proscuted a 5 your old men who first developed a lesdom an the right thenar antreso four god ODO-half years before the appearance of smell lesion on the dght Lower otramity which gradually increased in stzo end now ledes appeared from time to tone on the attranities and left buttock Finnerods (732) patient, 2 year old Humgerlen women, developed tho inital laslon, a hard red sodales, on the hateroposterior surface of the night arm. There werd two ladons och on the right and left thigh and left leg and ano auch on the right leg, shoulder and scalp Frost (83) develbed a year old man whose lesions appeared first on the hands, later on the legs, and subsequently on the tous, an and body Tho kertonus vero accompanied by odens of tho hands and feat Thero #25 also one losion on the right eyebrow and one on och or Webster (271) described A 77 your old men who had involvement of the right hand and, to a slight dogree, of the right forearm for ds years preceding his deat. Along with a relattveh few other similar cures, this indicates that, aside from the type origipally described by Kapod, the condthon may be encountered with a Anglo or e fow localbred ciborescence, frequently only como side of the body which remain for an alcoded period before entendon occurs A patient hastag a mell roso red spot on the left hand, which Lurgely disappeared on pressure, was reportod by Salhorst and Polano (99) The disease hud apparently remained stationary for about 18 years, after which there was a relatively rapid ortenslon, so that, 20 years after onget, lalong on both the hands ond fect were clinfoally and histologically typial of hapott's sarcoor hreibich (133) described a patient hay ing only two cutaneous lesion both on the Hght foot Braun and Stuffer (33) reported . uso from the clinical towards and moulages of Preboes, in which the ladoos weto almost entirely restricted to the right hand there being only slight 2 Raport's Sarcoma Involvement of the left, and one legion on another part of the body In Marzecki's (100 caso 1) patient, the discuse was still confined to the right hand and albow two years after onuet. Cattanoo (38) observed two patients, both having a doglo ledos, in oro caso on the right leg and to the other on the left leg. In the cause described by Sanear and Wien (232b) tho manifestations of the disease were restricted to the left foot and ankle. 2. Scalp, Face and Ean A 72 year old man who had multiplo mall tumors on the face was described by Stilham and Smith (250) A largo, hard, purplo tumor mas had been 9 Losloos oo the face resembling Iculanto podules. 10 Lesions on the face resembling loukemic nodules. acred from the night ade of tho noso sa year before. Threo years later a mall purplo mes bad appeared on tho Hght wist und dortumn of the left hand. Symment (251A) patient, A 18 year old man, bad vaguo gastrointestinal symptoms for soven vean and a small prurltlc growth on the auride of tho oght car A strnilar growth had been removed from the semo Arci e your prendously There were recurrent, unilateral lesson Ettology 23 in the aurico of the right ear without corresponding changes in other parts of the body A 65 your old man, described by Jessup (127) hed only two lesims; 000 on the scalp, the other on the forehead. These snall painless lessons had appeared four month before, and the scalp lesion was diagnosed as a cyat it was removed surgally but failed to heal and new levons contoved to appear The unibal laation in tho salp, without bocules or plaques elsewhere on the body made the clinical dlagacals 11 Module on the car terembling granulom pogrikeun difficult. Wise (55a) described a patient, hertog 2 Dodule on the pose wto also how another podulo on the cheek and lesions on the thighs, fect and forearms Vacle and apot laro (1556) described a patient with a podulo on the ear ud discrete podules on the fact. Pulkon (210) reported a 58 ye.ir old man who had a small purplish tumor on the outer badet Kaporls Sarcoma of the right uppet oyelid which had boen present for alght months. Soon, tuo smilar lesions developed in cloco appost Hon to the first, and similar lesions doveloped on the right cer and neck Fraser (81c) commentod that the histological diagnosis in this case is Kapool's worcoma in spite of tho - naval location of the lasloos. He mentioned that Ewing (682) observed a patient having a stoglo losion on the tongue Spłnno (215) reported a 67 year old man who doveloped en borttaton bchind the right ear dx months before. Soveral mall nodulas appeared a short time later blitchell. (171) patient, a H year old Italian, presented thickoned, infiltrated, inflammatory plaques and Dodules of 10 years duration au tho ears, nok, hands and feet. A patient described by Gilchrist and Ketron (87b cose 2) had se distinct leddons on the face Are of which, at first glance, appeared to bo chronio localized patches of rosacea. The majority of the ladoos wero present on tho lower third of tho Doso. The two lesions on the right sido wero semiglobular firm, raised, reddish browo nodules, about 4 mm. in diameter of tivo years duration. The three lesions on the left sido were deep rod, Anooth, flat, rather Incar thepoul elevations, docply Infiltrated on palpation, measuring 3 by 6 mms, and had been present for six to eight months There wero lesions on the center of tho cheek and chic present for one month, which were dark red, alightly raised, deeply infiltrated patches, 1 by Lk ons in stze, and shoired no loss of color opon promuro Tho wual history according to Croctor (49) begins with diffuse cenotic spots which pass into infiltrations and then Into nodules, with or without preceding odeme Traub (20) reported. 65 year old Italian who first presented a rather large purplish, ulcerativo Josion of the left ala nast, which wu 9 cmsm diameter and elevated and above the surface of the sidin Tbo Indons, present for four months, wero pe1- stred, pigmented and vascular and involved tho Hght car Ilp and dorul surface of the fingers of the left hand Dorfcl (58 cu 8) described a patient in wharn the disease first appeared on the cyelids ten years proviously with hemorrhages Ekologu 3 and nodulah Lalons subsequently appeared on the scalp and faco und later became geenitzelAnother patient (561010 14) doveloped leddons on the pope is yeen provionly and latar losions appeared on the fingers and toes. Epsteln (65) roported a patient who first doveloped per purlc lesions on tho right car A Dodulo then appeared on this spot which was soon followed by the development of two moro nodules close to the original lesion. Becker mod Thatcher (165 cme I) described a 52 year old man who was first soen Builta 12 Unilateral leloos va soalp and forehead Kaposi's Sarcoma with syphilite hemiplegle. Ho presented a fow dusky ory thematous, semi-globular tumors, up to 0.5 cms, lo diameter on the face and chin which bed been present for threo ar four years, and had recured after completo removal by electrodalocation. A 52 your old Swedish men, presented by Blucfarb (201) Arst doveloped a sodule on the left cur kven months before the appearance of nodulas on the left sido of the scalp and forehead corresponding to the ophthalmlo branch of the trigeminal nerve. In cases reported by Perrin (1960) and by Funk (81) tho Initial efflorescences were on the cheeks and nose. Hansson (108) dercribed ree patients havtog the initial turnor on the DOSO His first patient, a 4 year old man, had a tumor of the left nostal which had gradually locreased in sto for two Donths. The lexon bled occasionally but caused no other rymptoms. It was reddish, scab-encrusted and about tho sao of e pea Ho was unablo to find previous reports of such a primary localization. Kaposd and several other investigators definitely state that nodules appear on the face only at a much Liter stage of the disease. In a seria of 16 casesh Ronchese and Keru (210b ) noted that two patients had ledoos on the ears, one on the face and one on tho salp 3. Oral The mucous membrane of the prouth is beliciod to be one of the most common localizations in the more advanced stages of the disease, according to Philippson (107) Beto hordt (19) and Selle (230) The growths in tho vidblo mucous memberupe always appear to bo assocated with dmtlar cutaneou growths, and were it not for this wodation, might casus acape clinical klentication, according to Symmets (5) The palato, aspedalh the hard palate secma to be the most comglon dto of involvement in the mouth, although patients with hernorrhaglo lessons in the court paleto and guns were reported by Kingsbury (134) and Sequein (233c) Lesions occurring on tho bard palate as an elongated purplish plaque situated transverxels actors the roof of the mouth and invohing the posterior aspect of the hard palate, EHolow 77 wao decenbed by WYLE (2550) Kumezow (113) deknbed 1 patient with nodules on tho left side of the hard palato and 000 on the upper lip which wu tho itzo af e cherry At antopsy thero werd two per died bluish nodulas present at the base of the tongue. In the car descalbed by Boerman and Tavs (17) the patient had a purplish nodule on the left sido of the hard palate. Groes (101) patient hed lesions on the hard palato and lorer llp in addition to other lesions Down Log (37) also describod poch a more. The first manifestation of the disease Cured on the hard palato fin the case of 13. Drerete podules on the hard palate. Liposi o sarcoma of the bone reported by Fet (70) Two of Ilanssons (108) patient had ledoru bcalized on the face aos of whom also had blushered inoltrations in the hard palate Bluefarb and Rodin (286) abo obserial to patients havog lestons on the hard palate. Silent (291) presented a man who had an ulcerating og 29 Repod's Sarcoma szed man on the right toonil Feldmans (712) patient had a solitery laddon on tho herd paleto. Stats (246 caro 2) desolbed a petient with two 2mm bluish tumor nodules on the left side of the tongue, fust behind the sulcus terminale. 14 Group of onglomatow nodules torvotving the hard palato. 15 Close-up vow of hostration 14 Araus, et al (7) reported sodales on both lips, the palate and the dorsun of the tongue. Grzybowshd (102) reported in volvement of the tongue Roger and Vignos (208) patient had involvement of the lips and the patient described by Pearce Ehology 99 od Veller (183) had tovolvement of tho gums. This man developed tho discaso at the sto of an extracted tooth Cntaneous Dodules pon folowed. Zakons (872) patient bad leslocus on tho sort palato and left tondl. Wlso (2831) described a patient who had two puuplish Dodules on the left anterior pillar and on the edfolning hard palate. Stats (218 Caso 1) reported a por-sized, drcumscribed purplish Dodule on the buccal mucone. The 50 year old Italian man reported by Seagravo (28) had a mess in the nasopharynr. Confunctival nodules havo been reported in Kapods un coms and aro urually associated with histologically similar growths in other acceaublo macow membranes, such as tho mouth, pharynı and esophagues, and always it appean, with corresponding cutADCOUS changes. A patient reported by Symma (25.10) presented an oval new growth in the coo- Junctiva immediately adjacent to the upper and onter odgo of tho left cornea. This letion wu movable and non-plg mental Thero was a similar cutan.cow growth on tho tenporal sdo of tho left lower eyelid. The conjunctival lesion in this case is believed the only on the far recorded in tho English literature on Kaposi's sarcoma, and the only recurrent conjunctival growth so far described. Although the cutaneous lessons in a posts marcoma tend to becomo ymmetric to conjunctival Doduos thus far recordod huvo beto unilateral Schmidt (205) presented a 65 year old man with involve ment of the oropharytur and largar On the soft polato, fauce, and posterior pharynx, thero vue sharply marginated, pur plish red, maksed, adematous lesion. The epiglottis was thick med and involved by the same process; us was also tho base of the tongus, stiform smis, arytpiglotto fold, fuko cords od ventricular arcuSulzberger (33) reported a women who had a lesion in the left tonsillar pres Ocho (183) presented a 58 year old man who had lesions of the mouth. Dorld (56. case 7) desaibed a patient who had the typical distribution of the discuss u wel as mokement of the Toocot manbrina kapods Sarcoma Pautrlar eod Lexueur (1010) stato that lealous in the mouth are very raro and the particular form desenbed by then har pot been cincountered before. This patient presented vegeten dve tumor formation with ulcemtion, the lesions were paints und emulated, audio 4 Othor Artue of Inpolarmmt. A 60 year old Italian tan, described by Stratton (252) had pulo and swelling of the Bagets and toes for the preview dx months. The skin of these greu ww of a puplich red color and board ko moodstency studded here and there with elevated, hard, purpllab red 16 Pende lens of Repod's uncoma. 17 Penude loons of Lapos nitom 18. Peok levons of Kapous arcome (Cartety of Francesco Rondbeve Poststed ed., 14 101 1053) Etolog 31 podules Thero wao similar lesions behind and on the margins of the curr u well as on the penis. The scrotum xbowed . Angls, much larger elevated tumor of the same type, 15 by 9 comes in size, which had developed folosing severe prultus of thu arca tal yeans before. The right lnguinal lymph nodes WETO enlarged. Turbon of the atremitía end an bad och curred a few mooths after exporto to to cold in Aleske. There Wus Do ovldenco of tumor formation to the viscer. Two patieob having involvement of the penis were reported by Barringer and Dan (13) The Art had two submucus Dodules on tho pentle bead, and the second a round solidappearing cherry red growth on the corona. Tbo ladon re- Kembled carcinoin. The patient descrlbod by Bluefub and Webster (98c) had podnkes on the penis Van Clovo and Hellwig (265) reported a 58 year old American man who, four years pomidously noted a small puplo-liko lesion in tho left groin. The lesson became larger and painful to pres Ano and Anally receded to do wreb ume, leaving a pig entod scar Soon, however a milar lesion appeared in the Hght grain and numerous analles lesions appeared over the anterior surface of the catreunities Philippson (187) described patient who had tormowly svollen genitalla and mouplo tudor of the peols which Intafered with micturition Stats (2-16 case 9) patient had a pea sized podule near the aternal Dentus deurna (177) dextíbed a 75 year old man who had a uretheral stricture produced by Kaposi's wrcoma. Agostr (3) reported 50 year ol] women who had involvement of the gluteal region IV PATHOLOGY A. Pathogenets IX DE PRESENT state of our knowledge, it is fruitles to spcculato concerning the nature of tho etiology of Kaposi's mrcome. There are ncarly as many thcores regarding tho nature of the origin of this disease us there aro investigators in the fiel. Bredy the opinions may be classified in Avo major groups 1) neoplasm, 2) Infectious granuloma with neoplastic potentielities, 3) systemic vascular disease, 4) der fvation from nervo Hosue, and 5) reticuloendothlal hyper plosia Tho major dificulty in clasdfylog this particular typo of lesion is due to the fact that there is no unanimity concerning the origin of the tumor Fibroblasts, perithelial and codothelial cells, myoncunal structures of tho glomus and so ticuloendothelial elements aro among those considered by various writers u the starting point of tho now growth Most Investigators agree that the disease has a multicontric orgia, and that letions in the internal vicere are not mctestato lesion. As evidence they dito tho blourro locultations of tho internal Lisdoms, their appearance, to como casos prior to cutaneous manifestations, and finally the lack of invasho tendende soother point, upon which most investigatori Agreo i that it is an affection of vessels, particularly blood channels and also lymph channel Vost writers believe that tho cells of tho infiltrato may ariso from tho onclothelial lintog of the vessels but othens attributo their origlo to the advent'ta of the perftheilun, or tho pery vascular lymph speces os mentional by Philippson (197) 39 Pathology 33 Bernhardt (10) Schreimme (987) and other. The spodlo cells are bellovod, by the majordty to be of endothelial origin, other belove that they are derived from tho tdycattle, whilo still other regard then u connoctive time celle Pack (1965) suggests that they may be caused by plasma cell transforma Hon Starnberg (218) believes that they arise from the mus culads of the adveoutle, at least in uomo ches, and this opinion is shared by Gens (83) Fischl (78) md Perrin (1850) whilo few othan bellove they onlginate from the round call in the early pertraxcubar infiltrato In one cu Pautrlar and Dilu (1912) found that the cellular darination wus from the vascular neuromuscular ermords and Schwunian elements. They are convinced that the discapo be neurovascular dysgoods and a pseudourcan. This opinion is supported by the work of Vigno and Pedat (2006) and Hudelo, et oL but is disputed by Radaell (202) and by Tramgateno and Fittipaldi (250) Dorffel (58) to convinced that the affection is primarily a deoare of the ra ticuloendothelial systero, including a ditubonco to to modo crtoganetto function, which at time may terminato la true malignedcy but hesitate to call the disceso e trwa roticalo endothellode, Hu opinion be based on the obcrvation that tho lofilltration so pervascular lymphocytold and reticular and is derived from tho endothelial and adventtal calls and, Lc, on the presence of monocytoods and pigment. The conception that he posts turcome to a disorder of the reticuloendothelial system, and that it may be moro or los closely related to the hymphoblastomas," is mupported by the opinions of Nicolas and Favto (178) Puhr (201) Lano and Grerardod (113) Blucfarb ood Wchster (80) and allo by the fact that most parts of the reticuloendothelial system has been involved in DUTOSTOU cases. The morphological resemblance to the neuroarterial gloonus bus been mentioned by Pautler und Dila (191a) whilo tho Aoal suggestion, by Lang and Hosthofer (145) is that the condition is a systemic anglaikatosis. lontier it is the opinion of Slacke and Cipollaro (166) that hapods mercoms I caused by an unknown systemlo 34 Koponi's Sarcoma agent which attacks tho vascular apparatus, causing chronio hyperplastic inflammation and granulomny, and the histogo.cat is compatiblo with, and indistinguishable from that of a malignant now growth of peoplesom. Tho mafority of frvestigators, including Dillard and Wauman (55) and Dorffd (56) agreo that the sarcoma is produced, althoogh this opinion is not held by all. I Neoplastic Theory Adherents of the theory of neoplada aro divided us to whether tho tumor is benign or malignant and as to the origin and nature of the tumor cells It wu Kapoofs (132b) contention that the disease was hilstologically e mall roumd cell sarcoms, with the occadonal occurrence of characteristic spindlo cells. Ho thought it was a general disease because it begao at the ceno timo on both hands and foot. Unna (263) agreed with Kapod and classified the disease with the purcomato. Othen who have placed this discuro in the warcoma group Includo Jordm (199) Justus (130) Aletscherild (168) Pollitzer (1992) Balser et al. (10) Selhont and Polano (029) Lieberthal (151b) and Hartzell (111) Kuzeo (142) stated that we are dealing with a sor cometow endoperithelioma and that the complicated genesis of these now formations is related to . Jisturbanco in the balance between the endothellum and tho perlthellum of the capillary next of the corlum under tho influence of an unbrown factor In consequence of this, tho codoperithelium cuced, tho lots of pormal change and bulkls an la complete, though similar tintor Hostie gradually fnvolving more and moro of the connective therve element in the tunor growth until, o in tho older modules, it finally forins A sarcomo like tugor There are others who beline the disease is malignant, vascular cancer o un anglosarcoma, La, ano made up of con Dectivo tove elements which apparently originated from the Adventitia of the vessels Development of new blood vessels is also a merked feature Halls (105) found the proceu first developlog us an englome and later due to the proferation Pathology of the veicular perithelium, It took on the duracteristics of an angiocarcoma. Jo considered it a peculiar form of sarcoma Bonhardt (19) and IIaml and Resat (108) thought it was a malignant proliferation of perithelial colls. Hadsell (202) md Bertadtal (21b) believe the endothelial elements are the polot of origio of neoplastic proliferation of angkuatous types Sacha, et al (2171) stated that Lapos nama s nerer Uplo Inflammatory proces. New blood vesick and spendio cells are present to all sections, oven those from pinhead dred lesions of one week, duration. They believe that from the be ginning the discurou a potential wrcoma, that all latou do Doll Decorarlly terminato a neoplasms, and thet it may be considered # sykemic anglosarcomatosi. Aegetet and Prolo (2) expressed the opinion that the disease is a vascular cancer anglosarcome, and thery regard the secondary ctral lalons u tru metastases. This is controrv to the opłalons of most observers who have studied Kaposi's marcarla, for they regard the vixeral involvement a not metastatic, but of independent origio. Corrales and Vwaurreta (91) are among Investigators who have favored the theory of an angiomarcoma in the discer Leyt (150) summarizes the widence against the concept of hapoufs sarcina sexs and in favor of the autocht thonour, rather than the metastatic origin of the lesions, a 1) the absence of a primary focus that progresstvely murger, 2) the appearance of wtdet separated lessons in cropx, 3) the spontaneous muenion of sonne lesions, and *) the fact that Histakmical esamination may real very early stage of de romet In late appearing losions Occasionally hardves kuin may malergo malignant degeneration and then grow a true arcoma and ause metastaser It appean mwent that the tendency to exsider Kaposi's wincora as a true sarcoma is waning and this theory Goda tert fo rocates amg the more recent im estigators. Inectious Theory Proponent of the infectious granuloma theon huise in thet are the natural history of the dheuse which w frequentlı factures spontaneous regreson 38 hapost, Sarcoma which would be difficult to reconcile with malignant Dcoplasm (Cholsser and Ramsey: 10) To their diædvantago stands the fact that no ettological agent has over been demostrated despito extensivo bacteriological work, and that transfer of tho disenso to animal has never been definitely estah Jisbed, although atteropis have been made using overy commom laboratory animal and bird. The erstence of certain predisposing causes anxl ording factors is postulated by various writers to aplain a malignant chango lo e provlowly benign growth, a peoplastic trang formation on the part of an Infectlow granulome or tho do velopment of reticuloendothelial hyperplade, according to Cholster and Ramsey (40) These include trauma, chiftog or freezing of tho affected parts, alcoholism, drug sensitivity crysipelas, cellulitis, arteriosclerosis, syphills and acuto tofoc tous diseases, such as cholera, malarla, influenza and poeu monio The adherent of the infectious thoory support their arguments mostly on the Jinical behavior of the directo notably its preponderanco in certain countries, its location and symmotry and the long duration and tendency to involution Philppsou (197) beloved in the infectious nature of tho disease He stated the pathological changes aro a now formation of capillaries and spindbo cell tissoo. Tho virus, bo bo Koves, enters the blood after an lucubation period, wbore it may call forth either type of reaction, ie the spindle celor tho hem- or lymph-anglomatous type. Sequeira (233b) considered the pathological reaction to bo moro the nature of an Inflammatory reaction than a blastoma. In camination of a dozen cases, Fmper (811) could find no etdence of sarcoule The carliest chunges appeared to be in tho capillaries. He doted e marked proliferation of the cudo thellum with u infiltration of mononuclar cells und fibro blasts. Coses have been reported in which true murcuna de icloped in the course of the disease, but it does not follow that tho original condition wu i acoplean It is wel lonti that an inflammatory process may wodengo changes that and Pathology 37 in a malignant proces In some of the reported cures ATCOTA mtavened not on tho Lapos ledon, but apparently us an Independent derelopment. Ewing (66b) belloved that it is a gredulom,, which to cer tain predisposed subjects, begins to take on Dooplastic property Tho now growth in tho vtecers are probably not, ho setos, metastases, but arise from multiplo fod which wero originally buflammatory The final tumor is the prodlo cell marcome. Ho agreed that the late stages acquire neoplegic properties and glue nue to a spladlo cell sarcoma, but potatod out that the prodaminance of inflammatory signs indicatod on Infectlow odgin of granulomatous tasuo with special involver ment of the apillary endothelium. Veckoo and Cipollaro (1556) concurred with this opinion. Do Ankels (53) stutod that the condition is an intermediate between an Infoction (granoloose) und wrcoma. Homma (121) described a caro of Kapoor's arcoma in which tho tual histologic picture was complicated by the presence of large numbers of glant calls both of the forelga body and Langhans type. Those calls vero faterpreted as indicating an inflammatory origin of Kaposi's sarcoma Stimulating processes, which onlinarily produce inflas mator, affect may on rufidently prolonged application, to duce mallmancy according to Dillard and Weidman (55) They found fwgus products in the mescnterk eod gastro bepatic lymph nodes which bord mome resemblesco to Acborton Schoenleini, but there was no proof that these were the etiologke factor On the band's of their studies, they believo tho fungus they foond is not the cause of the disease but beliat it should be searched for in all are which como to autopsy Thev bebieno there is some goncal stanic influence which induces a dratation of the lymph and blood ctrels in the peripheral parts, with inflammatory agents entering later to produce the hiperplastic features of the strate they derrored to produce dmilar lesions in rabbit by feadtog thern culture of Acharloo Schbenlend and infectiog cultures Intraperiteocalh brut had to posithe results.
38 kapod's Sarcoma The patient reported by Brmstog (30) was a 61 year old Negro w bo first developed small cutancow fumors on both fectsaloo yeon before. These progressed to lavolve the catro Hght role and toe and the left tastep. A dlagoods of blastotny cods, provod histologically bad been modo seven your proviously Ho beloves this would have the most ploudblocs planation of Kaporda sarcoma doveloping on the background of an older Infectious process Animal inoculations with two of Kaposi's sarcoma havo produced lations only in ono instance. Justus (130) reported having injected into the back of a white moruso some apuston of a rapidly growing area from a cuto of Kaposi's sarcoma. He later observed collection of new farmed celle, aspecully around the arteries, in the lungs, tho heart and liver Ho suc ceeded, through eunuldfydog tho bidacys of tho tjected animal, to transmitting this disease through five generations of nico, and the arou tafected alurays sborrod tho lesions character istic of Kaport's marcome. Civatto (11) belfoved that he had e portivo inoculation in a chichen but later learned that anglomatous lesions occur in chicken at the ato of rnoculetons of the most varlel tumors. Santori (218) reported ixg ativo Inoculation results in the anterior chamber of tho Mo of a guinea pig Gilchris and Ketron (875) stated that rosult with an autogenous vaccino suggest that it is not auto Inoculible Grinspen (100) suggested e Altrablę vino nec tion which in turn stimulates the reticulendothelial system Dospite the typical microscopic evolution of auch Lapod sarcoma leon from an inflammation-liko phase through granulanatous phase into a trvo neopledo, no bacteriologoal ar viral agent has ever been isolated. Relooculation of patients with their own tumor tissue by Pack (188) and animal tooc ulations by habitt, et al (170) and by Choloset and Ramsey (10) failed to induce tumors at the sites of inoculation Coto and Crump (1) infected white rats, guinea pigs young cats and rabbits with pieces of tissue annuldons, intraperitoacell but hed do positive rerolol. Those, and other aperiments, bave somewhat nulled the concept that tho tunor is on infectious granuloma.
Pathology 29 3. Suatemko Vacular Dimas. The hypothesis accepiod by Home Observers in that same agent, the chemical, bacterial or fugal, uitacia tho Yakular tecluntan, curing pumarily infiammation and ectada. lacleod (188) considers the process to be a prolforation of agenfutng comiectivo destro with vascular dilatation, H. belleve that, whatever the initial cus of tho discese, it Ant attacia the blood vennel, causing a proliferation of the fixed cells in its one of reaction. Ho stata that it had more the appearance of proliferating connective taste than a sarcoma and still loss does it resemble a granska The disease beglas as an angioma and the tumor stage to rolu from proliferation of connoctive tusue and endothelium, kocording to Gilchrut and Entrou (875) Their studies showed that the lessons originate in the skin, due to proliferation and dilatation of the blood capillaries, which are very frall at first and table to rupture This is followed by a prohferation of the totestitial connective time and endotheliaun, which grad ully obstcrates the blood space forming rolld tumont In the carly stages they resemblo in seno area, young connective Unse in others, marcomata As the lion grow oklet they ESSURSE * tre fiberos mport, and may undergo imobition Corastent with the formation of the humors, there u arclerosit of the anal arteries supplying them, causing . gradual de creare in the amoumt of blood. To this is due most likely txt Later eluton u well u involution. Gruber (102) statal that the lesions are not granu lomus brut beigu tupat. They are sentially angiomus, artsing by pollination of alrcady edisting blood vessels. Al though exlsthella proliferation occurs, these lesions amot be chiunfed with the makesthelionas because there are not the anh celb which proucrato morcover the rulothidal cells change their character to old ksion and become histiocytes or fibroblasts. The teri perithelioma is also one to be added, be beling On the other harxh, it is not a sarcoma, visce the proliferating cell show the dom of malignuncy but on the catran team to ungo kletosh Crnbowski cooclodet.
10 Kaposi's SarcoTAG Nicolas eod Favro (178) and Lang and Haxlhofer (1:45) favor the theory that the disease is a systerno anglamators and Ramel (203) classified the condition as an endothelioma. Santori (218) expressed the opinion that the underlying discoso is a congenital or acquired general debility of tho viscular system. Sternberg (218) studled tho podules in the submucosa of the Intestinos in one case and found the dov formation corresponded in overy way to the surrounding muscular coat. Ho considered the disease, pathologically a comblaabon of lymph end benengiectades associated with proliferation of the enooth musclo Hipics. Serm (231) concluded that Kapood's wrcome to a hauto-englo-endotheHom and that the accompanying round cell proliferation is merely a secondary reactivo pberomanca Kapool's wrcoma arises from capillary endothellum and is Dot a flbroblastic sarcona, according to Fraser (81b) Highman (1196) stated that many investigators believe that Abro blast are dorivod from codothelial Ancestors and this was also his opinion. After careful histological studies, Vovers and Jacobson (167) concluded that the condition is probably one of multiplo hemanglamatosis with a Abrosarcomatous change In the stroma of many of the tumor nodules. RadAcH (202) and Bertocdni (21b) believed tho endothelial elements were the point of origin of neoplastic pro Hferation of angiomatous type. It is the opinion of Bertacchi (212) that the growth originates from the codothelium of tho blood vessels. He bases this opinion on the similarity of the cell to crdothelal cells and the transition forms between the mure of cell and fully formed blood vessels, and also on the tubular and concentric atingement of the cells. Ho (316) emphasized the abdominal appearance of the blool capilloric, which displayed thickness and hyalinosis of the wall of both la prorimity to, and at a distance from the lealog Along this une hno is the crplanation of Leigheb (1.18) in e cuoio which Kaposi's urcome apparently followed an cythama from arsphenemine La desaibing a familla telangiectatic durplasia of the Oales Pathology typs Weber (2732) alluded to the pogiblo analogy and ar vocation of tho telanglectatic condition of thoshtn and mucow membranes of tho nose and mouth with certain telengloctatic conditions of the stomach, intestine, kidneys and lungs. Tho telangiectasta to this discuse bo rogarded u due to a conganital dovelopment dysplasia of tho small vessels potentially present at birth though aftea pot menlfesting itself until after puberty The possibility that a malignant vmecular growth originates from a localtra debility in the muscular system L shown by u mumba of reports in the literaturo, Coburn and Morgan (43) suggested that the condition to primarily a polyvucullus, followod by mulu focul benign anglomatosis, and that the proces may be dmilar to that which occurs in polyarteritis padon. Dertoed from Maroe Tlam. As early as 1904 Perrla (1911) emphastood the nervo lesions in Kapool's woon. Thle dieeuro wus regarded by Pantrier and Dios (1918) to bo a dysgenesis of the vessel and of their bouromuscular adpexA oo tho ono band, and of the nerves and their Schvenian clements, on the other They stress the pain and the disturb ance of local temperaturo observed and consider tho vncular and nerve lealous as tho matomophynological basis of the disease. Heatologically they observed, not arcane, but or genic cleulents to proliferation-resrels with a neuromuscular slove and a nerve network, and prolifaration of cells of Schorum with formation of tactilo corpuscles of Vagner Alcoher type. They believe that Kapod, murcoma wu to the vascular system what you Rollingharen disease is to the DCT US system. Hodele, et oL (10%) presented cuso which thoy allod Kaposi's telonglactatic peeudo-parcamatostAfter detailed histologlo studies thoy concluded that the lodon was not a coms but an englanatods combined with a proliferation of tho Schraon cells, that is, a dyxembryoplastie lesion of the cutaneous towels and of the adjacent Schwan element dit Llar to von Redlinghausens disease. IoLater report, Hodelo mod Cedliau (122) We of the opinion that the prolifera Kaposi's sarcoma Hon of tho neural cell is the autential and initial factor of the ledon, together with vascular deoformation arising from the proliferation of imitated codothelial colle They bellovo tho wholo process to be duo acudvely to the proliferation of sympathetic pertvascular nervo Abors As a result of Inflammation (perhaps duo to infection) tho nervo cells of tho media and adventita of normal vessels can first retrogress, then prohforato and becomo similar to the non-myelinated fibers of veghboring mosses. They believe that such lesions Aro hyperplastic, but not neoplastis, and point out that tho proudosarcomatosis of Kapor presents striktog analogies to von Rechlinghausen s disease They consider that the nervo elements in the vessel walls, irritated by tho circulating tarins may Initiate the turnor process in the vessels and connectivo tigruo which eventuata in Kapool's mercome. However they pointed out, no traces, or insuffident traces of tho Wagner Afelespet bollos, belloved by Pautrier and Dius (191b) to be the starting point of this processo, preto found to belog this about and the normal perve clements in the vertel walls may be capable of originating this tumor It was pointed out by Lano and Greenwood (1413) that some of the most compotent pathologists in this country while agreeing with Vasson : (163) arginal work on melanomas, do not consider the cases of other Pautrlor and Die (1919) or Hudclo a al. (129) to have been proved Becker and Thatcher (156) despite the use of servo stalna, were unablo to confirm these findings Anatomic changes in the central nervous syrtem or trophic disturbances with changes in the peripheral nervous system, were found by Semedow (231) Anal Sephier (219) They be llero there niets a relationship between Kaposi's sarcoma and the nervous syaten without pretedding that the new growth of this condition are Dove thosuo At tud wtopsies, Semeno found podules in the shen stoonach und intestíocs, liver pan- Ceas und Hunor, and the spinal ganglion was filled with pigment and a swelling of the neurogla pueld and profera tion of the connective tesite in the peripheral nerves. Ho Pathology believe the dance to be related to changes to the trophlo nerves, induced, to como ases, by the action of cold and opoara Saphier noted tho discaso spread aloog the periphcral Tye of the skin. Noul (191) believes that Lapod's morcone is caused by a fundamental aberrant Lintahlllty of the series fupplying tho blood vessels. Interferenco with the normal drauation of the blood ru considered respondblo for the datation of the terinim veel and the subsequent diapoulosis Tho later caplary proliferation wu interpreted in greament with Poutler and Dla (1912) concept of peurogenic cellular Clements Astfonifalaion of Reticulorndothelle Spurn Hyper plado. Thaco be consider the conditioa merely a reticuloeodothelial hyperplasia pote the suggestive htatological pfc toro of tho Jeoso and tho smultaneous OCCHIITEDCO of widely cattered lesioon, as well as the sot uncommon occurrence of other reticuloendothelial synon discases in patients having Kapols sarcame. There and reports descrbing variation from mild lymphocytosts to frank lymphatic leukemld, mycode fum soldes and lymphomarcoma. Association with mycods fungoules wu described by Lano and Greenwood (113) Lapoins (1416) and Wider (253 o 4) with Hodgkos diretso by Gilchrist and Ketron (875) Coldschlag (90) Talbort (258) Greenstein and Conston (98) Osbome, et al. (187 as 7) VcCarthy and Pack (133) Erf (00) and Wolf (987) with hmphorarana by llcCarth arrel Puck (153) Belloni (18) Hugins (118) md Blucfarb and exter (28) with IT phatic leukemnla bv Cole and Crump (H) Ilume anal Du pont (123) Sache and Grey (2176) and Fracter in Chikon (74) oo with myclad kuldemla by Teleschi col (257) and Cuiktet and Gulst (51) Thh asociation was also notal by Ells (81) Crop and Betton (97) husacmany (IC) and Warthin (970) The sites of the lessons further supports the hypothesis of retk uborndobelil origin, doce the region, mont kachly supplied with these Harry are most frequently insohnd On the bads of his study and corroboratlit nderke Ropouts Sarcoma found in tho literature, Dorffel (58) believes that Laports sarcoma is a disease of the retkuloendothelial system, a priori, not e true blastama, bat which may in some cases, become ano aftu long continued imitation. The reticuloendothelial origin of the disease is aloo supported by Dalle Favore (52) Puhr (201) Lane and Greenwood (143) Dupoot (689) Dil- Land and Weldman (55) and others. That tho products of blood degeneration night act as the endothelial stimulus hus been suggested (55) Bodo marrow cells in a caso of Kaposs prcoma WeTO studied by Sutch, et al (251) who concluded that tho cells were of reticuloendothelial origin. Becker and Thatcher (15b) also condder the disease as one of tho reticulocndothelial tyrstem, with a disturbanco in the monocytogenic function. They agrce with Dorffel (58) that the presence of hemorrhage pro- Wiferation of elements of the blood vessel walls, and cellular Infiltration along with monocytor's fustifies thus theory Tho lymphocytoid cells of the infiltrate, which Dorffel conglilarod reticuloendothelial cells, may forin spindlo cells giving a pic turo resembling lattico fibers, they state. Tho presence of Lattico Abers throughout tho Inoltrato supports thus theory Further ovidence of gweralized reticuloendothelial dysplasu Les offered by Stats (216) and Tedeschi, et al. (257) wbo obketved curly microscopic deviations in the bono marrow lymph node, spleen and liver which were distinct from the primary cutaneous changes. McCarthy and Pack (153) ako observed dmilar microscopic changes. Puht (201) aplained the formation of blood vessels in the growth by fusion and subsequent evacuation of cells in which bo rccognized all the functional and morphological character istics of the reticuloendothchal cells. Creppi and Bettoni (97) rached a strnilar concludon in studying an unusual caso of Leposti cercoma with atensive visceral manifestations and concomitant bemolytic splenomegaly The involvement of tho reticlocudothelial system in this case, they belore, had manifested trell in the splenic area with an craggeration of tho hemocatheretic function proper to this organ, while else Pathology where it had given thao to Dooplastic growth with the char acterleta of Esport's sarcane Cuocbono (101) Flare (77) Choducer esd Remedy (10) and Goldenith (82) also cardfy Capoul's WrCocos Among the diseases of the reticuloendothellel syuton Tedechl, st al (257) stato that Kapood's sarcoma busy teunic in nature zod Whely to lavalve independently and in a predictablo menner simultanovaly ar pucedvely the skin And the internal organs and that it is cherocterized fran tho histologle stendpotot by a great verlety of pattern inherent to the multiplo dovelopmental potentialities of the basis uult of growth, the reticulorn call In a detailed hatologic report, Dorffel (58) dorcibed the endothelial cells which originally line the dous broaldng away to forin solld metres ad Anally becoming spindled to ro- Scrable fibroblasts. Ho found that the cellular follitrute displays a surprising chances of forms to which the term lympho cytold as wood by Blarchand (150) is most applicablo. Ho beloves that the cella artso from tho reticuloendothelial syutem and gives the following resons: ) the celle aro distribated almost entirely about the blood vodeb, 2) tho veure wall cloenta aro svolla, md there is definito ovider.co of proliferation, 3) toy form a reticului, present abo tu porno places in the stoel walls thengelves, thus allowing banor rages by dissection (Herzog 116) to occur 4) there is a multiplicity of cell forms with many transitional cuangos, # they are differentiated from the central veerel to the periphery of the nodule, and 8) Largo endothelial cells are found con pected to the tattue of the Year, but are just on the verge of catering the systemic drculation and becoming trondtional cells, or are already definitely monocytos. These trandtional cells mai monocytos are found also in the times a well as in the croulating blood. The mooocytas represent the third system of blood prodoctop in the body the other two betog lytophogeneous and mychogenours, according to Scintog (22) Tho monocytos be is convinced ate from the endothelial cells. Dorfid (58) plasia of spindlo collo arising in tho perithchal tips, without neoplasm originating in the perithelial tissuo from emlry and mesenchymal cells (lymphocytodd cells of Alarchudd) which result in a type of cell growth uniquo for this dbeuge After a period of years, the cells may undargo malgrat deret tlou produdng true marcome, resulting in metaste and deutli. Truo moteutes is raro although the spindo cold which constituto the tumor may becomno malgrant and are rise to tnio sau coma u in Bocher and Thatches (155 cure ) und a roportod by Eving (685) Dillard and leden (55) 170 Abroblast determine the growth behavior of arch of Purce may be charactatud Amonling to Symmen (251A) 1) the fibroblast may product Kaposi's Sarcoma 1 Pamsey 10, Dorffel 58, and Pautrlar od Dos 15 (11) er Theoria Do Amics (53) who, in 1597 bad.com we cases of Kaposi's sarcoing than any other brett Trebeved that the relation of the disease to crowd (ne and tho primary lesion must be comodadd a typo w to itself lying between a granuloma and a smern het and Thatcher (155) studied embryonal mesenchy goed 10 tissue cultures and found many histiocytos ubice und greatly from the fusiform cells. The latter Hero Dot mbroblasts and they did not act as malignant cells da They Autul that there is a unique typo of cell reactions of the mhiryonic cells in Laposi's sarcoma The lymphocy told cele of Marchand aro stimulated by some unknown agent whool may produce a benign neoplasm. They believe that Kapoor surcome is a benign neoplasm. In some instances it goes are occasionally goes on to the formation of arcome. They belieto to malignant hyperplasia, just as myofibrome of the other one that tho cact classification of the tumor is too heardous to attempt, but it is probably a benign deoplasm with a multice tre origin characterized by a vascular proliferation and hypert dugnating the particular coll of the soveral types constitutung the peritbellum. The most logical emplenation of Keports Aircoma, in their optolon, is that it is a multicentric berning Dorffel (58) and Nackeo and Cipollaro (150) thu pliases by which Kapol's Pathology 15 when it bed given the to u neoplastic growth with the chur acteristic of Kapods ATCOTA. Guccaro (101) Fleres (77) Cholbar and Ramsey (40) and Goldomith (89) eloo candy Kapod's arcome among the disues of the reticuloendothelial syam. Tedeschel, o al (257) state that Kaposi's surcons by tentc fn nature and likely to involve independently and in an unpredictable manner simalmocously or succedvely the skin and the internal organs and that it is charactertood from the lustologic #undpoint by a great vadoty of patterns inherent to the moltiplo developmental potentialities of tho basic unit of growth, tho reticulum cell. In a detailed blatologic report, Doufel (58) described tho endothelial cells which alginally Hino tho snus broaldng away to form sold masses and fmally becoming pindled to romanble filbgoblasts. Ho found that the cellular infiltrato di play a surpriang dichoes of forms to which the term lymphocytold, as wed by Alarchand (150) is most applicable. Ho believes that these cells orire from tho reticuloendothelial mytom and gives tho following regions 1) the cell are distrib uted almost entirely about the blood vessel, 2) the veel all elements are rollen, and there is definite evidence of proliferation, 3) they form a reticum, present also to como places in tho veerel walls themselyn, thus elowing benor rages by dissection (Herzog 118) to occur 4) there is a moltiplictty of cell forts with many transitional changes, ou they are differentiated from the central retsel to the periphery of the module, and 5) largo endothelial cells are found coo Docted to the tottma of the vessel, but ero fust on the vergo of catering the systemio drculation and becoming transitional cells, or are already definitely monocytes. These transitional cells and monocytes are found also in the Norues u well as to the circulatiog blood. The tronocyte represent tho third system of blood produc Bon in the body the other two being lymphogeneous and orologeoons according to Schilling (292) The Dronocytes, he is convinced, arise from the codothelial cells Dorfd (58) 16 Kaport's Sorcomra found evidence of codothelal proliferation with mopocytes in tho Hauos and monocytes to the differential blood scar In this manner Fischer Wassel's (75) roqutrement & fulfilled since he states that when o tumor has originated from proliferating endothelial cells, monocytosis should be present. Tho presence of gitterfasern is perhaps further evidence of tho fnvolvement of the reticuloendothelial ryger, once they are usually found, us described by Way and Slovekom (372) and others, and confined by Dorel (58) as demonstrated 10 cloarly in the early anglomatous stogo of Kaposi's sarcoma Gitterfasern are also found normally in the liver (Nosslo.211) Whato cell of the reticuloendothelial system aro so nunerous Another manifestation is found in the presence of tho pod ules of snus-hko space Ited by endothelial cells containing fine pigment grunnles and strongly resembling slnilar cells and spaces in the spleen, according to Dorffel (50) Ho be Heves that tho cell typo involved basically is tho pluripotent cell of the reticuloendothelial system which, owing to its various developmental potentialites, is able to give rise to comply and unpredictable structares. Ho corkcludes that Kaposi's frcoma is a disease of the reticulocndothelial system, including a disturbance of its promocytogenetic function, wbich at tunes may taminato in true malignancy Ho support this belief with the following potpts of ovidence 1) the vascular system throughout the body is the site of definite clinical changes such as ectaste, varicosities and purpuric lesions ) Dodules may be found distributed along the vessels and ad herent to the vessels, 3) microscopically newly formed capil laries and a polymorphou cellular infiltrate are found the cell of which artse from the reticuloendothelial system (lymphocytoid cells) 4) the monocyte system is afectod as evidenced by an increasel number of Donocyta in the torud and monocytosis to the clifferential blood acer and 5) tho presence of gitterfaseti docs pot permit too definite conclu dons on this point. Walzer (2061) stated that many cases of keposts marcos and in sarcoma or some other form of Jymphoblastoma and Pathology thard to probably e definito relationship between theso dirouren. The conception of tho systemic nature of Kapod s prcoma and its relation to lymphoblastome and reticuloendothelioane bave been roceutly emphasized. Dupont (58) noted tho fulform cells which constitutod most of the timer man, their phagocytic properties and their Ability to form roxettes of reticulum flbers, and he belloved that only ono call, the histiocyte, Lepable of such activity Ho proposed tho vamo'ngiarettculose." It wus suggested by Chotixer and Romsey (40) that all tho multiplo cell type fouod might ato from the reticuloendothellal system and, therefore, thought the condition might bo delgoated angloreticolocndothellama." Grinspen (100) suggested a systemic reticuloendothelial byperplada duo tovina infection and stated that the discuro should rightly be called englo-reticuloendothelios's of Kaposl Tho roticuloendothellal theory wus disputed by Ewing (88b) who bekored that Lapoel marcome was a mysterio disento in which e moro specific structure, ruch u tho neuromytwarterial glomla was lovolved. Lren (130) doubted that the cells primarily involved to the reticuloendothelial cells and stated that the only substantial patnts in favor of this origin the presence of celle provided with phagocytic power in tho powly formed nodules According to DlcCarthy and Pack (153) slution of the problem of pathogered is not imminent and powably will not materialtze until the cultures of arly Kapood's tumors are favestignted. They bellers that although a start has been medo in this direction, the result yo not condudve und tistue cultures would recin to implicato primith • merenchymal denuo o the origin. It is neccesary to determine which body times are capable of glutag rise to the numrows and diverse structura observed to Dewly formed blood vessels, including endothelium ad dietitial connective tissue, embryoolo splodlo cells, lympbold clements and well organized reticulum. Only from the retic ulococotchol system could all these type be derived (Cbols- 18 Kapod's Sarcoma Set And Ramsey 10 Dorffel 58, and Pantrier and Dls 1892) 8 Other Tkzorlu. Do Amicis (53) who, in 1897 had crumined moro cases of spod's sarcoma than any other investi gator believed that the relation of the discuso to arcoma was a close one, and tho primery lesion most be conddared a typo peculiar to itself, lying between a granuloma and I marcoma. Becher and Thatcher (156) studlod anbryonal mesenchymal call in Haruo cultures and found many histiocytes which differed greatly from the fudform celle The latter vero Dot Abroblasts, and they did not act as malignant cello do. Thoy stated that there is a unique type of cell reaction of tho embryonic cells in Lepost's urcoma. The lymphocytold cela of Varchand ero stimulated by some unknown agent which may produce a bonigo peoplasm They bellovo that Lepoals flrcoma ts e benigu neoplam. In romo instances it goch an to malignant hyperplasie, just as myofibroma of the uterus occasionally goes on to the formation of mercome. Thoy ballero that the cuoct classification of the tumor I too hamardow to attempt, but it is probably a benigo Deoplasm with a multicen tric origin, characterized by a vecular proliferation and hypkt plasia of spindlo cells, aridng in the perithelial tissue, without designattog the particular cell of tho xeytral types constituting the perithelium. The most logical asplanation of Laposi's carcotu, to their opinion, is that it is a multicentric benign Dcoplas originating in tho parlthelial tiesuo from embryonic mesenchymal cell (lymphocy told cells of Varchand) which results in a type of cell growth unique for this discoso After a period of years the colls may undergo malignant degenket ation, producing true marcoma, resulting in octastasis and death. True metastasis is rare Although the spadlo cells which constitute the tumor may become malignant and give rise to a truc tarona a In Becker and Thatchers (15b caso ) cuo and u reported by Ewing (88b) Dillard and Weldmed (55) Dorffel (58) and Vacker and Cipollaro (155b) Tho Abroblast determines the growth behavior of each of the phases by which Loposis arcana may bo characterized, According to Symmet (231a) 1) the ibroblast may produco 6 Pathology young argentophilic collagenow Abrils which renforce the maturo collagenow bundle in such a manncs es to aid in the process of bauling ) the fibroblast may maintain a low almost Hagnant capacity for growth over a lang number of years, and 3) the fibroblast may puddenly asumo activo mellgrant properties and terminato life by widespread destruction of toves through the process of metastad. Hetologlo study of the younger growth, according to Symmas, shows that from the onset two opposing factou uro operative ono tending to balog about replacement of tho sodules by tho production of collagenow Abrils, the other tending to malotain the capodty of tho podalo to grow but at an extremely slow rate. Exclud tog ruch incidental features u bemorrhaglo atraventious between the fibroblasts and deposits of pigment following dostruction of the rod blood cells, tho histologic appearance of the well developed growths to Kaposi's arcoma is scarcely to be distinguished from that of spadlo cell arcom. Wbercu tho spindho call arcoma ahport always grows rapidly and without restratot, the progress of the spindle coll growths of Kapods Arcome ! retarded almost to the polot of stagnation. In other words, the growth of Kaposi's sarcoma aro histo logically malignant but cltaically benign. The process of spoon taneous regression and healing to the cutaneous lesions of Kaposto arcoma, SymDe coattoued, is probably instituted by the sudden relauso, traumatic or otherwte, of largo por bers of red blood cells followed by lajury to or destruction of Largo Deighboring fibroblasts and finalh by connectio theo Teplacement The maller and presumably younger Dodule no permeated by a couples network of argentophilic retic ulion which serves pulmonly us a orechnical support for tho Bbroblasts Pick (198a) bellard that spindle cell true had its origin to the plasma col which accompany the blood vessel. These plasma cells he stated are capable of dertloping into endo the Halble cell that after ctbalted tergy coded to Abrow tissue He classed the disease with rootd grow the ilstologic Miks at autopsy utte done br Dalla Favera Kapad's Sarcoma (52) in two cases having sodules in tho trachea, bronchi, limgs, stomach and Intestines, lymph nodes and skio. Ho be- Heved that spindlo cell odginated to the connecthie tatue and In the cutaneous lastom, u able to trace the gradual chango of the spindlo cell bundles into tho colagenow tanio of tho corlum end to demonstrato fibrils in the spindle cells. In tho lungs, the mall pinheed sized podules had developed almost entirely around the small bronchl and tho accoonpanying branches of the pulmonary arteries. Ho au of the opinion that the acting course of the discuso might call forth both types of reaction spontaneouhy Len the per formation of capillaries and tho spadlo cell growth The quantitativo roaction between the two was verlablo but the anglomatous tisruo become less pronounced as the lesions grow older Belloni (18) condders tho discaso a hyperplastic mesanchy mal ono with multiple, systematically arranged foch, pro senting the following cheracteristics A Pronounced poullon blastic tendency Loy a capacity for various types of anatomofunction differentiation (1 Angloblastic, predominatiog to tho regions of tho cutaneous und intestinal Lestons, 9 Fibroblasta, ausing the datrical regresdon of the lesions, 3. Vectophagicoperlc anal Flematopotetic lympho-monocytic type of cells, Flarer s cells) and B Tho possibility of a leukemic and peoplastic development Although a solution of the etiology u far from gettled, recept advances to the general deld of carcinogenests make it plausible now to suspect somo syetemic carcinogen acting on the vucular Hasties as a posufblo agent, according to VcCarthy and Pack (153) Suche hypothesis, they belkeve, would more logically emplain the distinctive teodency of Kaposi's sarcoma to invoke the leg bilateralh and smuretrally and ultimate h manifest itself throughout the viscera with multiple adjoinion tumors in the inflammatory granulomatous eod arcomatous phase and resembling multicentric foci rather than metastores It is now possible to induce similar anglosarcomes in animals with Injections of chemical cardnogens Andervont (5) and White and Steirert (277) have induced 51 Pathology Dancom betegn mod malignant englornatous tumors, several of which actod u primary tuon of multicentric origin, with the administration of ozo compounds and hydrocarbons to mice. Andervont also detcunipod that femalo mice were far moro nasceptible to such woduction than male mics, and clear established the loftuence of scor hormones by markedly reduceg loduction with crustration and testosterone pellet moplasta. Furtha evidence of a syrenic factor in humans by tho auty prollferitive change of Lapoofs arcoma in the the spleen and lymph nodes which are far remord from the grow and obvious frcomatous nodules to the skin, as descubed by State (248) and Teachd, at (957) There are probably the etiological factors fuch as box bor Danes, since the incidence of Laport's morcoma is high in menu Among 1,058 patients with bemanghome, reviewed by Watson and McCarthy (571) 65 per cent were women. They also noted defolto growth stimulation of engloons at tho onset of pregnancy and the menarche. Many woman with Kapod's Aroma hana mapliested it most btwarto forins. A fulminang Cose dacnbed by Symmen (2534) ww en laten woman who arred for mly 34 days ofter the cuteneous lesions appeared A 83 year old woman wbo developed an allergic artritis following sensitivity to peudellin was descrlbed by aIcCinn, et al (184) She then developed a vascular (Kapoor's) mar corna. Coburn and Ilorun (43) tuggested that this condition is petonarily a polyvasculitis, followed by multi-focal benign anglomatosis. They also suggested that the process may be sontlar to that which runt fn parlarteritis nodos. B. Vorbid natomy Lsco (139) states, "The ardinal changes that churcterian Kapods Larcoma bistologically consig of new growth and etoda of capillarices, lemontage and proliferation of copped tie thee The vasculo changes concern pronally the blood vessels and only to alene ertest, the lymph vascular rysten.
Kaposto Sarcoma Pigmentation develops from hemortuge, and reaction proc esres occur about vascular and connoctivo Hogue, finally scach ing the stago of lovalation and healing with leddons resembling scar tissue. Novertheless, the histologlo pictures uro very differ ent, depending on whether vascular or fibrous changes pro dominate in the todo und whether drcumscribed or dufuso cellular proliferation develops This brief Introduction presents tho essential foatures pros cat to Kaposi's sarcoma. It is important when studytorg the pathology that lesions of vadows ages bo carefully cumloed, for not only do losioms vary in different cases, but they also very tn tndividual cases. Singlo sections may be anglonnatous, granulometow or neoplastic, or they may show combinations of any of these histological fogtures The cutis is the sto of development of the ledon. Tho spidemen's plavi do primary rolo in the process and the ep! dermal changes such us thinndog, acanthosis, hyperheratosis or breaking down are all secondary to the advancing procesi The first change consists of capillary dlatation and apillary budding Dorffel (56) and Van Clove and Hellwig (285) bowcvit stated that the first pathologło change is the occur race of hemorrhage in the cuts from one or many varels at different depths in the cutis. Thoy believe the hemorrhage to the initial, pathologic featuro of the disease, together with tho proliferation of the vessel wall clapent and cellular in Altration. The vessels in the vanity show cagorgement and some dilatation, and there is already present a deposit of Iron rig meat Clinicalls at this stage of the hemorlugo is deep, both Ing is found grooly other than a faint hemoorthagle moculo. If the harsotthago is more superficial, localized areas of blood atravaution lo the skin, or estn e diffuse purpur-Ilo eruptron, may be observed. This stage must be one of short dura too because in most cases evidence of cellular Infiltratioa already present at the time of examination. Dorffel (58) be Less that infiltration of the round cells occur almost smal tercously with the honorluge On the other hand, Sachs, et al (217a) believe that the
Pathology
533
10. Anpomatous, purvulomatous ad scoplastic bag present to
ape hytologlo sectioa.
* Inftrato consisting of spondlo celke, hemontage pigroot and
nonly formed aquilles
51 Kaposi's Sarcoma hemorrhago is secondary rather than primary and is the result of erythrocytes wandering into the surrounding tissue from imperfectly formed nety vessels or through ruptured or damaged vessels. The probferation of thin waled vessels is thus to be considered us the primary process and hemortugo is secondary 'The newly formod ectatio capilaries appear to be Anglonus and these vessels lio ha cellular stromra composed of hymphocytes, pluma celle, kundering connectivo de colla, angloblasts and mast cells. Betireen the bundles of capillarlos there are delicate, spindlaliho fibroblasti. Deponding on which of the above featnes predominato, tho soction may appear to bo anglomatou, sbrous gmpulomatous, inflammatory or highly maugnant morcom Hansson (109) states that there is agrcement that the malo distinctive features of both tumors and infiltrations is the new growth and dilatation of capillaries. Many of tho nests are to a largo extent composed of ach capillarfez Besides this capillary proliferation, there also occurs, almost without cu ception, beinarthages and deposits of blood pigment in the surrounding tloue. Philippcon (197) Sellet (330) and Plck (1080) have also reported dilatation of the lymph capillantes Because of the increased vascularity the new best sometimes resemble Inflammatory granulation tissto In additioo to tho newly formed cplurics, there is often a fair amount of furocellular polymorphism present, which gives rise to carcoma Ilo structures, especially in lesions of long standing. Hemorrhages are probably respowsible for outritional dh turbances which sometimes rosult in pectosis and dautz tion of both tumors and inftrations. Spontan cow healing of the single nests nav tako placo in this manner os noted by Hansson (109) Sclerosis of tho nests occurs following radite tlon therapy He camined tho tumors histologically beforo and after radiation in three cases, and in all tho sclerotic con diton w. very distinct As the inAltrute decreases the vessel wall cells reveal thetr abuilty to form Dow blood vessels, according to Dorffel (58) Houtil this inability has been impairal, os ovidenced by
Pathology
55
Irupillin dilat twn ud badding
2 Engry minit of blood sexel und deposition of tro płpreal
Rapost's Sorcomo the presenco of incompletely formed blood vessels In these pour blood vessels there is also proferation of the vessel all elements Vackoo and Cipolero (1556) dte Satenstein (200) regarding the threo patbologoal processes recognized inflammatory granulomatous and neoplastic. By this ho means that the very carly stage resembles Inflammation and may be yo clandled. Later stages may resemblo sbronte hyperplastia Inflammation, granuloma or a now growth. Very often these processes overlap and produce a borderline or mlred picture When the inflammatory proces predomiaates, tho carlinal features aro: I Dilatation of blood vessels and lymphatics 2. Pertvascular Infiltration of mind cells-round cells, con rectivo turo cells and some plasmu cells 3. Edeme, hetborrbago and fron-containtog pigment When the granulomatous element is accentuated, the pre dominaung features aro I Proliferation of conn.cctive tisne with young nucleated cells predominating and perivascular Infiltration of mixed cell . Ederna, hemorrhago und Iron pigment When the neoplastic feature predominates the picture vurles with tho elements forvolved: 1. When the changes aro primarily vascular the picture may be that of angloma or may resemble granulation Moto or granulomo 2. When the lymphatics are involved, the picture may suggest lymphangloma or a mixture of angioma and lymphanglome 3. When the connectivo tione proliferation predominates fibroma, eogtofibrome, anglosercome, or spfadlo cell sar come may be suggested The three Hage of development of Kaposi's sarcoma art detelbed by Handi and Pleset (108) In the first stags the
Pathology
57
Newly formed ectatik aptarles
24 Indammitory page of Lapod's TOMA,
Kapod's Sarcoms
Arcon
25 Inflammatory stage of Kaposi's
28. Coulomatou stage of eposu s vrcome
Pathology
97 G
lumat ws toy of Wapos um (husbet magniſation
of Fraue 28)
ilk & of hupi wcuor Lor pon a mapification
Rapods Sarcoma
1
* Fibroblasts and yocular proliferation Perlyuxular hmpho
te in Altration
30. Diffuse vuculitis luulo prolifantoo Parlvascular hy mopbo
guc infltraboo
81
Pathology
11
31 Fibroblasts
32 Palglapdular Iniltrate.
Kapods Sarcoma early growth are falatly vidblo to the nahel oyd and barely palpablo. Alictoscopically the blood vessels of the shin aro surrounded by zones of celle rich in protoplas retembling epithclold cells Often, these cells show a completo series of transitional forms, indicating thetr transformation into con Dective tissue Alany of the blood vessels break up into a fino network of namifying capillarlos composed solely of a wall of andothelum Tho neoplasm in this stage is dmilar to fibroangloma In the second stage, the growth are clinically promfrent as elevated red or purplo Intradermic tuman. Histolog feally they are poorly delimited growths composed of aggrogates of fustform cells with a perivascular distribution. The blood vessels are numerous and vary in oza. Vany red blood cells are to be found in the interstitial tesuch In tho thin stage, the older growths sarme a dark brown or bluish color depending on the extent of hemoglobin destruction. In somno of the more advanced cases, tho cellular aggregates are found about the terminations of the peripheral nerves, tho meat glands, the pilounotor muscles and hair roots, and even in the subcutanoow adipose tive. Tho fact that tbecso cellular foc! rarely fuse connotas an independent and spontaneou origin for each of them, for a general arcomatosis of this type is not produced by the emigration of cells. Before any of the features show pronounced proliferation, the findings are thoso described in the inflammatory stage, according to Saches, et al (2172) The blood vessels of the upper and mid cutis are dilated, increased in number frequently Aded with blood elements and arranged in groups or scattered diffusely throughout the cutis. The endothellal cells are swallon and have lange vesicular nude projecting into the lumen. These nuclel may be round and woowhat hyperchromatic rather than ovel and vesicular Instead of being clongated, the cells may appear rectangular (crosssection of the endothelial cell) and resemblo somewhat the cmbryonc pluma cell of the young granulation tissue Theo lymph vessels and spaces we prominent The cellular Infiltratton sparte, petivasculu u well as diffuse and composcu Pathology of wall round and wondering connective tissue cells, Angloblesta and some sphodla cells. Plasma and most cells aro o cosocally seen. There is no hyperplasia of connectiva tempo ood no changes in the elastic true. Erythrocytes or intru cellular or extracellular pranules of heroosiderin, bodicates bonorrhage. As tho process dovelops (lato stogo: granulomatous ar Dicoplastic) they coutinue difcrent pictures un noted us vascular connective tisste or cellular hyperplasia becomes prominent. With the proliferation of vessels, the appearancois that of angioma. Vascular and cellolar hyperplasla may mimic granulomas, glomus tumor and granuloma pyogenicum. If the Verrels are associated with mumgToUs Angloblasts, Kapoli par coma may stimulate mglosarcome, pelthelloma or endothchone. In como of the sections, thero werd areas that a no way differed from planus turgor Whero spindlo cell prolifer atlon is the characteristic feature, the rerult is a spidlo cell arcoma. Planes of cells ated in all directions, and at times show mitotic figures, other cellular clements, as well u vas cular and counoctivo tissue hyperplada, are not prominent. If the connectivo tovo, rather than cellular clementi, ts in Trosedl, the picture suggest an anglofibroma Tho growth of Allrous tatue is never extensive and frequently sparates tho proces into lobules Frequently areas characteristio of vart ou dermatoves are seen in the same section. Vascular hypa plase, betonthage engloblasts and spindlo cells are found throughout the evolution of the directo eccept perhaps in the development of spindle cel arcoole, where the proliferation of indle cells overshadows all other features As the lesions become older according to Daffel (56) tho lympbou told ekments begin to play a losser part and the Anglomatour features become more promisest. The lympbotold cells, howeit may Latet go od to form spindle-liko cella, to pie the picture of moneiho lesions, which he be- Herm represents the final stage of the proces. Ho has seen auch a trandhon am in the earliest kedon, place be studied tho polymorphism of the cells from the ressel to tho periphery of the codulo where the amoume tho splodle-like fort and 61 Kaporis Sarcoma products in some place, thin collagen Abers. At times the soglomatous features ao so typical that dlagoods of cavett ou hemangioma would be fustified. Again, in cases in which the activity of the reticuloendothelial system is more marked, the anglomatous foetiro uro present along with wrcoma UILO features with mitotic figures, and the diagnosis suggested would bo anglosarcome. In such cases of lacrcured activity ho found tho previously described, but so important poly morphism of the cellular infiltrates the wholo picturo repro penting all stages of development of the process in the ledon Dorffel (50) belloves that the relationship between the presence or absence of gitterfasern and elastic fiber is of foterort In the onllest lesion, the elastic toto is injured, or perhaps ovan completely destroyed throughout tho wholo cutle, even includtog parts of the cutis where tho actual leston u not present At this thone, gitterfasetu aro very prominont As tho lesion dovelops and the anglomatous picturo appear, the gitterfasero remain a stalking feature, whilo tho clastic tissue, although completely destroyed within the tumor may be found lets severely tofured to the adjacent tissue. In the All later carcome-like stage there aro very fow gitterfasom to bo found, and still no clastie tissue is present in the tumor There is, however an almost DOTMAI elastica in tho tlaucs Derr tho turnors. The collagen fibers in the earliest lesions may bo tho sto of degenerative changes of edema, bomogenization and alight vacuoltration, in some places, sccording to Dorffel (58) With the van Glcsod stain, bright pink, dah formed collagen fibcts make up tho stroma of the tumorous structure, whllo the fibers in the adjacent tissue weer normal. In the later sur como lhe stage with the extremely celular make-up of tho tumor few collagen Abct are found, where in the surround ing thats the collagen Sbers sbow regredie changes, which ultimately will be supplanted by Det granulation tisstie. From this description, one must be struck by the great pourts pour sessed by the retkulocaclothelial cells to form och eclis other than those that they produce under konnal conditions le
Pathology
13. Inut do not nti huma thes on puulomatous stage (Cute
fWTO)
34 P mun of celluler lo Attrate
80 Lapas Sarcoina believes that cells, to have such power co be only embryonal in the sense that they are un differentiated and remeln 50 until need for their differentiation arises, os meu tood by Herzog (116) Alarchand (159) Vestmon (184) and others Large amounts of fron pigment are present in the cutis which may be intracellular as within vessel wall alls or in the cell of tho in Altrate, according to Dorffel (58) However much of the pigment i atracellular giving the cath a strong ly podtive from (beznosiderin) reaction throughout the in Altrato occur in a diffuso form, with a tendency to bo perivascular It consists mostly of plasma cell, fled wandertog colls and cell of the lymphocytle group Endothelial cells, in direct relation to blood vessels or entirely apart from them, sto seen groupod in the infiltrato, proliferativo changes being constant. Vost cell lymphoblasts and transitional cells oro also found in small nun bcts scattered throughout the infltrato. Elastic Abers are partly or completely destroyed, the collagen Albers are unnlly involvel, but to only a slight degrco and the fat and nerve stains show no pathological changes. Extension of the lessons is along the vessels and by periplicral outcastoo around the yousels. Vlachce and apollard (155b) reported that pigment granules coull ocrodonally be seen fn the cell. They did not observo mclanin although this has beep reported by llamedi and Rent (108) Rottman (214) Vasson (162) and a fent other. Cens (85) and Ireo (19) believe that in such fostances the diagnosis was CITODCous, the discuse belog associated with loulomla or mycosis fungoldes, or that the presence of the pigment was due to arsenic therapy There is almost univerzal absence of melanin in Lapors sarcoma, c1 cept in the epidermis. The late changes are moro numerous and Yurial, according to Vackee and Cipollaro (1555) The Infiltrato spreads and forms masses of nodales, consisting of cells of vartous types small round cells, pleama cells, connective Hipuo cells, spirullo cells and, occasionally mast cells and gant cells. The glant Pathology 87 cells appear to be of the foreign body type. The formation of dew capillaries is a prominent feature There are often Dumer otus, cctatic and even cavernous. They give rise to hemorrhages and subsequent pigmcntary deporte Tho connecthe tissue becomes hyperplastic and often degenerated Elastic tissue and the appendages diuppear As they noted, any of these features may prolominate, o many of them may occur in various parts of a anglo specimen. The atensive infiltrato 33. Pads morphero o celular tafiltrate often courists mosth of spindle cells with whorl formation and oumcrous mitotic figures. Levolution is bulkcated by a large oumber of plasma cells, poor staining properties, victolization and other sidence of Jegedention, together with Ebrotic transformation, producing the appearance of chronic minulation tissue with ultimate regeneration, fibrosis or com urtzation. 1 this has been reported in oklet kestoos by prac tically all Imestigaton, but Dorffd (58) voted widcoce of beginning tosolution men in very early lesions. He found that signs of involution may be ruggested non lo early Raport's Sarcoma pepules, but in later stages are quito pronounced. Ho de Kiibed thoro Agos u: poorly stalning cells, vacualization of mudel, cytoplasm or both, and some fragmenteton and die integration of nuclet Rarely small areas of myzomatous de generation could be found. Kapod (132b) suggested that fbrin appoared in the Lirgo vessels and resulted in clotting of red blood celk, thus causing fovalution of tho alder tumor, and this was corroborated by Daniel. Small amounts of fibrin weto present in come vessels in any us well as to late tumor, even when signs of involution were abrent, but in auch minimal amounts that it was scarcely coacetablo that this could result in involution of the tumort. In conjunction with theso degenerative changes, Abrow transformation of parts of the tumor with the appearance of chronic granulation tissue was found Perhaps this granulation there is evidence of the final function of the vessel wall cell that have been able to retain their normal capacity to form regeneration tisso in the body Dorffel concludes. Tbcto lo much difference of optaton regarding tho gonesha of spindo cells Somo (Soquein 233b) Attributo their pres- Ence to proliferating andothelial cell. However most tovestigators, including Dalla Favera (52) bellovo they originate from cells of the connectivo tlusta Sternberg (218) considers that thieso colls arise from mooth musclo fibert Spiadlo cells Aro found only in spindle cell percome, or in a proces which may oventuato in such a arcome, according to Sachs, et al (2172) Thoy uro lod to believe that tho sarcomatow proces Is pot due to a simple accumulation of spindlo cells from tho perent cells, but may depeodcm proliferation of these cells from other spindle cells. The numerous mitotic figuras found In these alls would substantlato this view Tho cytoplasm of the splodle cells is scant and the cell tapety to polet at other end the nucleu i nartor oval ani vestcular with loosely eitunged and Hghtly stalped chromatin Tho kogth of the cell | ipprodmately tutce that of the nucleus Ther doubt that this cell plays any role in the production of cot legen. Vany different types of celk, even epithelial cell, may Pathology 60 have spadlo shapes, such cells should be referred to not as spindle cells, but as spindle-shaped cells. They Hate that the relation of the fibroblast to the spindle cell d un now Cootrury to the opinion of many they bellevo that the two cells ero different in morphology to function and probably in derivation The fibroblast is much larger in all dimendons and often Las falilo projection extending from the taperiog pofats It has an oval vescalar nuclety which is largo and greater in diameter than the nucleus of the spindlo cell Un Le tho spodle cell, the fibroblast does foren collagen. 'The angloblast is beloved to arlo from the endothelial cells and cemetimo referred to us en adothelotd cell lexo An Bloon (162) bellovel that the term angioblast should not bue wed However whilo the term andothettotd cell may bo acceptable, Sache, dal prefer to rotatn the name angloblast becaino these cells Aro mold to give rise to pow vessels and because the mature cel usually does not reseblo an andothelial oell. The engloblett be a round puoleu approxt mately one and core-half to two times tho siso of tho nucleu of a lymphocyte It stalns deeply but the chromatin doa not appear as a soll man As a rule, little or do cytoplaan obserred. However a fow of their section showed the nogloblunts with considerable stoplean. This is not apliko Uxo polyhedral pavement like appearance of endothelial ces when the flat surface devamined. In suman Lapoods arame is often codied as early or Late and u Inflammatory granulomatos or peoplastic. Such diutton is not entirely accurate for the pathologic plo ture does not always coincide with the duration of the process. While such a classification arbitrary it to coveolant and often helps to correlate the pathological picture with tho dhnical picture Thie im estigators disagree on the patho genesis, thero lo almost completo accord as to the charac teristic histological features. The histological structore comits of the toAltrate, numerous spindle shaped cells, bemontage pigment and Dewly formed pullarlas and blood spacci.
V SYMPTOMS A. Subjecthe Symptoms SUBC UDJECTIVE Symptoms aused by the cutaneous manifesta tions of Lapods Arcoma ure lowally slight or absent. Patients having numerous Large Lesions on the atremities, particularly those with marked edema, may have local discomfort and interference with, or even loss of function of these parts Visceral involvement may cause bemorrhage, diarrhea and other constitutional symptoms, tocluding emacation. There is occasionally hemorrhage from the cutaneous orifces, par tlaularly the Dose mouth and throat. There may be itching and burning, us described by Vleyers and Jacobson (167) Tiro of Sytumers (P5laicores fond 5) patients had intense and persistent prudtus of the affected areas and Dorffel (58) described prints and cdemna which preceded the eruption. This intense pruritus is very unusual In kaposli sarcoma and as a rule, is not a major complaint. As a result of the pruritus, trauma may cause bleeding of the lesions. Occadonally there is complaint of pain. This is uwalh, duo to the localization of nodules on the fect, which causes palo op walking This symptom a present in four patients reported by Hansson (108) and was described by Feldman (716) and Shonet (240) Schiller et al (21) presented 1 67 year old man who complained of cokl feet and keys, and numerous small, shin colored and hemorrhagic growths on the doram of the feet There bad been some degree of shin discoloration for about 10 years, and recently sharp pains had occurred in the area of discoloration 70
Symptomus
71
38. Sculer ladod.
37 Incular lesioa.
72 Kapod's Sarcoma B. Objective Symptoms The initial manifestations may bo e macuk, nodulo, plaque, in area of Infiltration, or even purpur. The initial lesion usually appear us a reddened, well demarcated macule. The early maculo progresses and becomes elevated, the color darhens and bluish red nodules or plaques appear Frequent ly both macules and podulas aro preunt, but occasionally the cruption consists of one or the other alone, or one type may predominate. с 38 Alecular lesions 30 Nodular lesions 1 Edoma. This symptom su probably overlooked by Kaposd, since it is not mentionod in his otherwiso cxhaustivo description of the disease. The orplanation probably lies in the fact that hla patients were avamined when tho disolso was far avanced, at which time it is auctptional to find e casc without multiple Dodukes and Infiltrations Phillipson (197) was the first to desalbe alone ou a sngle manifestation Edone of the atremities is common in Kaposi's warcoma. It may be the first manifestation of the disease and may procede any other lesions by mooths or years, as in Stats (2.10 DO 1) patient, and those reported by Dalla Faitha (52) Symptoms 73 Mierzedt (189) and Hmon (108) In other instances well tug may ocar late in the cours of the deve several types of odernu may occur Stats states that it may take the form of putting edena, to many ways nipaidally similar to the ederne men in other diseases auch as congestive heart falltre. It is 10. Plequoi lato usually symmetrial and is somewhat alleviated by elevation of tho part There is sometimes muual tendency to tarmimato sharply below the ince; the foot and leg being encral time larte than normal in the while the knee and thigh are of nome proportions, us in two of his patient (216 casos lnd 3) At that times the thigh i also invohad in the process ud a similar picture has also been obectved 78 hapod's Sercoma tumors do not appear until a relathely late stage. This edem, in association with Kaposi's sarcoma, was first described by Pick (1882) Hanssons patients, both women, hed estdence of hmophanglectatc elephantiasts, and one patient had les varicositles which had caused discomfort for twenty years Little is known regarding the course of this type of disease and the diagnosis is often dificult. The following case (103) Ilustrates the difficulties encountered by both the clinican und pathologist. This patient, . 10 year old woman, bud, 17 years previously addenly developed marled cdema of tho leg. pehts and right lablum majora in the course of a few darz This edema did not subddo and tad years later was dlagnosed o elephantlass, plastic surgery was performed with no inprovement. Nine years later sul small cutaneous tunsors appeared on the lower leg which wcto at first shin-colorrd and Later becamo reddish-blue. This condition was diagnosed as Brooms and amputation of the leg about the knee was per formed Histologic examination revealed vascular carcom which tho pathologist believed to be malignant and also considerable clema of the tissues Eight years later several pea sized, reddish blue cutaneous tumors appeared between the toes of the left foot The histological diagnosis of these Leslom wu Kaposis arcoma. Treatment consisted of 15 mg radium applied to the tumors for two hours. The kedons diminished ud paled in color and at the final cumination yours after onset of the disease, she was found to be free of symptoms 1 llemorthage Dilated blood vessels (telangiectado) mas at times, be viable in the nodules and planures or in appannuy normal skin, according to VacKee and Cipolaro (1555) Petocluso and purpura are occasionally present The actor reace of localized and widespread purpure with the de velopment of noduks and plaques has been reported by a Dumber of obreros, among them, Philliprison (197) Verdes da Costa (165) Aaposi (12A) Dillaru and Iduman (53) Havas (119) od Pelagatti (194) Symptoms Rayout and Cachera (205) described 1 65 year old man with Kapool's parcoma asociated with atelts of the lower a tremities end e purpuric syndrome. The disceso was of 18 moothe duration. Rocutgen cremination disclosed etenstvo caldication of the artela of the legAlthough he had no extensivo benoorhages, numerous petechias Hero present on the legs, the blooding thuo was prolonged and the blood clot Doprotracto Philippson (197) emphastzed that hemorrhage, Infiltration about the blood vessels, proliferation of tho vowel wall cloments and formation of new blood vessels wae manifestations of the influence of a todn which he believed w doro to un unkoowo microorganion. His first patient had typical oodules of Lapord's marcoma for six months when a generalized, bemorhagic, purpurle Hko cruption addenly appeared. Somo hemorrhagle spot receded entirely but others vero replaced by new typical podules Approximately stx months elapsed between tho appcerrace of the bemorrhagic spots and the appouran.co of fully developed nodules in tho samo urta Three moaths later another bemontugle eruption, Hmited to the Lower extremities, appeared, later still, another generalized eruption appeared. Each cuacabation of hemorrhagle leziona wu folloved by the development of some new nodular Tho mccood patient had a shunilar wuption tovolving both ky od tho left forcarm which also gne rise to Dew Dodules. These berrorhages were an unfavorablo sign, however once typical nodules son appeared to these vel. The 78 year old man described by Slendes da Costa (165) had atravesations of blood on both lower atremities fol lowed by typical modules of Kaposi's sarcoma in these areas. He concluded that a travestion of blood is the earliest dgn of the discre. Dillard and Wedman (55) favored the conception that hemorrhage ku a poloury factor to the production of these kuont. The believe that hemorrhage may be secondary to such factors u etposture to coll arteriosclerod, or ergotium, uod, u the blood didntegrated the substance producod
78
Kaposi's Sarcoma
tul
13. lieportages of the toe
H Haponibager of the too and legs.
Symptoms 79 might, in some cases, be irritating to the endothelial cells od act a stimuli to the proliferatio However Lapoal (1382) candered hemanthagens e solatta ly Lato symptom it u poolblo that be abseived this pho DO01001 without altachtag a great deal of dgang tot poco ho zatlonol the development of Armatous nodules In arcus of preceding catmcou banonbage in one caso Tho presence of oumzow apall nodules within a largo lewatige on tho thigh was observed by Dalle Favera () Others who bevo descolhed berorluga ve Hayas (112) Fonk (81) Koeboer (1371) Bernhardt (10) Riehl (207) Pelagata (191) Pick (1988) Aloriand (180) Brinn and why * Lymph pona libe tumen kostety of Friency Ronctiese 80 Kaposi's Sarcoma Seuffes (98) aod Sevenon (231) Hemontage from the Dose, mouth, totestinal tract and lungs, have also been doxribed by SomeLOW (231) Paolini (189) and Brann and Scuffer (28) Changes analogous to thoso in the blood vessels are found less frequently in tho lymphatic vessels (210c) Such observations wero rocorded by Pick (1982) who doscribed laryo lymphatic cysts on tho artremities which becamo slightly hemorrhagic and later doveloped into typical Kapost nodules These lymphatic cyst did not always become hemor ragic sometimes typical nodules developed directly from clear cysts A hemorrhagic tendency was described by Vlackro add apollaro (1556) Dalle Favera (52) Andrews (6) and Denzet and Leopold (51) The degree of edema occadon ally affected, to some atent, by local hemorrhagc, according to Stats (246) This occurs in the form of largo Aubcutancows bematomas, purpure, or bleeding of a mucomal tumor Infroqucnth thero may be a generalized purpurlo stato The his tology of the Kaposi lesion plains como of these manifesta tons. Hemorrhago is the usual microscopic Araling. It orig Inater in the telangiectases and, once it occurs in close prox imits to nodules or plaques, some cutaneous bleeding can be attributed to these lesions. In other cases, purpura appcan on apparently normal skdo. During this resorption of hematomes, clusters of Kapod nodules may appear in an area of fading purpur2, as mentioned by Dorffel (56) In the few cases to which studies have been made, no abnormalities of the blood ing or clotting mechanism which would account for those btzerte manifestations were found Operathe wounds do not bleed etcostuively to these patients and tho mucows membruno bleeding is apparenth always due to betriorthago from a Lepost Dodule according to State Etredve cpuste us or bleeding from the gums has not bec noted. llc (218 cose 3) described a 13 year old woman whose illness begao 11 years beforo with edema of both legs and a painless, colorless swellfog near the Hight ankle Eight years lato a smiler but Wuish swelling, appeared on the left ankle and numerou, painless,
Limplungoma ho tumom. (Coutesy of Francesco Ronchosc,
VD)
likeratol letoos of hapoul's urcoma
89 Raport's Sarcoma bluish nodules then appeared on the lower extremities, ac companied by widoproud spontancow ecchymoses and progressivo weakness. 3. Modulus. Kaposi (132) described the classical loslans es nodillos "ranging from the size of a pepper corn to that of . pa or a hazel nut, and brownish red or bluish red in color appear in the sun without apparent counc, either local or general. They are smooth on the surface, elastlo in consistency and very often resemblo blood blister. They may occur singly or in plaque-bike group when in patches, the central portion of the induration is degenerated and gives Hic to a discolored depression. The nodules generally develop first on the soles or the dorsum of the foot, thercefter spread ing rapidly to the hande Alost of than appear on the parti. Durlog the course of the disease, single or multiplo tumors also appear although in lesser Dunbers, on the legs, arms, face And truk The modules sometimes atrophy and may ulcerato at a later stage Finally stuntlar tumor develop in tho mucous membranes of the mouth and in the mucous membranes of the ventricles and the intestines. The lesions may be decrete coalescent, and even cooglom cate, according to Vlachee and Cipollero (153) Occasionally they are closely crowded togethe forming Largo and mall masses or tumors. They are firm, often shiny and rarely translucent The Dodules range from pio-bead to bedo atze or larger and there may be only two or three lesions, or they may be numerous. Tho nodules, round or oval in shape arc about? ang in diameter Thoy aro risal, flat, decph plg mented, or of a brownish or purplish red color markedly indurated. The disease appears to cxtend dceph toto the corlum. Babes (8) described one patient who had 150 lasions. The lesions wually occur in Duluples, not infrequcoth in dozens, sometimes in hundreels, and morely to one or two according to Symmets (251) Even to the carlier stages of adlution, the modules are recognizable and when the discare is well developed there are reddish or purplah nodules, plaquos un plateau-like formations. I sulk the goalket Symptoms nodulea ero rounded, the plaques present a flat or podolated sanfuce, and the platescu Ithe growths aro Irregular nodular mu undulating. The individual nodules may vary from a few Ione, to 2 or 3 cm in diameter whilo tbe plaques and plateau-Biko granthu may reach coormou proportions, very ting from 5 to 20 cms. or more. The podules do not appear to coplesce; tho pleques od pletenu appear to represent the apansion of multplo large and smell nodules, closely apposed to one another Another tearLable clinical featuro of Kapoor's Arcome, according to Symmer, is the frequency with which attain of the maller podules lose their reddith a purplish appearance and becomo yellowish, fatntly spelled by red or sometimes by light green. They deappear gradually without Pance, leaving upooth whitish or binowalsh scan to bo replaced in time by Dow Dodales in the immediate vicinity or at a distance from the old. As the disease progresses, tho collagenow tissues to the vicinity of these podules becomno thicmellymph studs ocain and the skin is thrown into folds so that the atremity presents much the emo appourance a that involved by the Dooparndtic form of clephantieda Final ly tho growths may ulovate, usually as a result of traini, Rond Moontime produco superidal cacarlettatus and, at other times, opet letons of fornldablo dimensions Tho patient presented by Orinsby add Mitchell (1882) bed Folkd oderne of the legs and feet. Vany podulos, varytog from a po to a wall colo fo size, wero distributed over theso ucut There were leddons on the buttocla and one on the glans poole. Wile (279) commented on the large number of onell rolular lesions, which had not coulctcod to forno plaques and which he believed to be somewhat unusual lo rare instances the disesto may appear fn the form of tumors from the onset (the demblee type) v mentioned by Burccellato (32) + Ulceration of Modules. Although not frequent, ulcera tion of nodules may occur As a role lesions on the upper citronlikes do bot ulcerate while those on the lower extreto- 81 Kapod's Sarcoma 18 cerated lesions of Kaposis 01. ities ulcerate as a result of hmphedema and vascular obliter atlon Ulceration of the modules was desobed by Hansson (108 case 1) in patient with soveral small blush red slight painful tumors on the dorsal aspects of the hunds and feet These lesions entouch, Increased in size and a tumor of the left foot began to ulcctate After roentgen ray therapy all Symptoms 85 tho tumon diappeared. Howeyet rocetteDco of the lastaus on the darrum of the foot pacasdated anopptation, 4 85 year old men wbo developed ladoms on the back of the hands ten years before, wus presented by Wiro (985) Recently a group of dimostred plaques, which undervrent involution, hed appeared on the palm. In the discussion of thu cese, Fox (802) stated that ulceration was an unmal feature, inco Kaposi's arcoMDa b characteriad by lack of plecration, and the small size of the lardons la his opłalan, large tumors do not occur as to mycosis fungoides. Bernstens (20) patient had a boardlike, blachdeh brown InAlltration of the night leg with ulceration, pigmented nodules in the ares, and many Infiltrated pigmented spots an tho entire trunk, with some infiltrations om other parts of tho body Tho cutancow changes had previously been regarded as varicose vela stasis dermatitis, and the ulcerations were ascribed to an infury Bunch (31) commented on a 30 your okl man who developed lesions on the dght foot, followed by Leron on the internal melloohes, and later on the left foot and ankle. The lesions were first brownish, then purple, and then became indurated. They became alocrated and painful o the dorsum of the record and third toet of the left foot A 58 yet old man who doeloped a hard nodule on the back of tho haod wpa reported by Ochs (183) Shortly after a clskog of this lesions, Dodules appeared on the hands and fect, and on lesions on the bid of the right hand became uker atel. Two patients having ucuration of the turnons weto reported by Ronchose and Kern (2100) The patient presented by Weyers and Jacobson (167) Wie an 82 year old American who developed puntas and burning along the medial forface of the right leg. One month later a mall podule appeared just below the Loee, grew rapidly and was soonctimes tender and puinful Thu lesion later ulcer atel and bled rather cash Aparent who first developed kesions on the left foot and leg 20 yean prolowly was reported by Dorff (50 cose 12) Later lertom appeared ao the left arm ond hand luch were ulcerating and fungatio 68 haposés Sercoma Ulceration, in the carly stages of Laport's sarcoma, may closely resemblo stass ulcers, according to Farbet ct al. (89) One of their patients required amputation of the leg, and the second patient obtained relief of pain and apparent resolution of the cutaneous leslang following rontgen ray therapy 5. Plaqua and Infiltration. The plaques are will faish well marginated and mango in size from a dime, or smaller to an adult hand or larget They may be round, oval or irreg ular in shape. At first there may bo оnh slight infiltration, which becomes more pronounced as the lesian grow outlet Not infrequently a combination of infltrading plaques and discrete nodules produce an atendie hard selling of the 19 Plaques and inflrations. Coutes of Frukesco Roostese Posgrad ved ) 50 Cutaneous pientation of the feet and key resembling Schambergs diseise Symptom 87 hands, feet, and of legs which may attoin the proportions of clephantiasia Tho entiro autancos surfaco may become fnydhed, accord Ing to Funk (81) Tho sha of the aittiities, buttock, and abdomen, as well as the subcutaneous tous, because Huse ly tiltrated, of bourd lilo condstency and Immovable, with an even surface which is nodular und a dark violet brown (plun) color He found that sourly all patients had plateliko depressions in ltalatod Dodules and th otro groups of podule w a result of central resorption, whilo occadonally many noduks dimappeared entirely At doxes, the cutaneous ledon acquiro a locally infltrativo chancter agruming the spect of plaques, to produce a rough, bound-tho swelling of one or more atremties, and marked enlargement may result Tluruo augmentation arribed to tumor Infiltration was reported by Vlocheo and Cipollaro (1656) Acgetter and Poulo (9) and Ormuby and llontgom 51 Cutubcous pentation of the too resembling Schambery : decise 88 kapott's Sarcoma ay (1806) There may also be blockage of lymph drainage either by local infiltration in the lymphatic or regional ob struction at the trunk Thickening of collagenous tigue areas adjacent to the sidn nodules may further impedo lymph dreinage and accelerato the swoling Goldschlag (90) and Dillerd and Weldoen (55) commented on Localized sclerodermic change in the extremities and trunk us a cause of swelling and e similar process aus noted by Stats (216 caso 3) Thero may also bo more or loss Widespread, red blue lo Altrations in the skin, according to Hanson (108) Lo the tunars they are often localbred on the trenuties, although in mare ad vanced stages of the disease, they may also appear on the truck face and mucou mezabranes of the mouth. He found thesc Infiltrations, as well as tumors, present in sit of his patkota. A year old man, presented by Oppenheim (165) how symmetrical bluth spots and infltrated plaques with a mmooth surface involving both sols, tho Lateral aspects of both feet and over the anlla. 6 Unusual Banifestations Varicouties and Unuudl Pigmentations Vany bizarre fca turut may be proxat in tho carly stage of kupoai's sarcoma. Not infrequently the onset of the disease la manifested by a pigmentary disturbance resembling Schambergs discuss, en to presexo of varicose vem, particularly in young adulte Vascular changes may appear as varicosities with tumors distributed along the vessels and fixed to them, according to Dorffel (50) In 1899 Fuak (81) first notal the presence of VOTIOW ectasta and collal, cnlarged veins Deus the trumon. To same year lwpost (139) presented a typical cue with dilted veins of the loua citremities. Distribution of Kaposi carcoma nodules along the reous systern wu also noted by S. Uc (230) Philipson (187) H.Je (103) Sethont and Polado (99) Varland (180) Dalla Farera (52) Ehrmann (01) Brunn and Sculler (29) and by Hledge (114) Tad patients who descloped scaling, pruritic patches around the ankles were described by Farbet et al (29) Both hol Symptoms 80 mild venous insufficiency and, after soveral months, devcloped dinically and histopathologically recognizablo Kapoo's ar come. These cases illustrato bow Lapod's sarcoma, in its carly stages, may simulato stads dermatitis and studs ukeration of the legs. Pigmiitattoo atending to tho plantar aspect of the foot and dorsum of the toca frequently develops Stasis dermatitis Keldon, If over produce pigmentation in these areas Palpa tion of the individual katonai Kaposi's sarcoma reveals to duration. Stass dermatite and ukeration may be associated with raibcr marked sclaons of the skin in the affected area and around the anklos, but indurated nodules are not present Perrin (1995) states that not infrequently the disease boging with purplo discoloration in diffuso patcher, very often rymetrical, on the hands and feet and begins moro aften on the floor than on the extensor surfaces Burckhardt (33) reported a 13 year okl men who had red, palaful and purluc meculo-popolar lordons on the role of the lect. A patient described by Shoblo (241) was a 74 ycer old man with septic thrombophlebitis of the Hght leg which resolved readily with administration of penicilin. There wu un cupton, of at least the years duration, on the back of both wibits, de feot and legs. The lesions, moro or less tymmetrical, were nodular and macular disetele, oval in shape, and varied between to ex cms in diameter their surfaco was smooth and shiny This cause Illustrates that well marked celem of the keys does not necesarily occur only in the more advanced stages of Kaposi's sarcoma. In the majority of cuso It be an earh smplom lo discussing this case, Lemberger (149) decibed a patient whose onk sytoptoms were hema turla und bilateral, wel marked cleme Three or four small blutsh spous wite Later noted on both legs and within two seurs time the condition descloped to be Kaposi's sarcoma Ilo lead thet edema y almost invariably present and is often a very prominent symptom of this disease Patients having atasie thrombophlebitis have been reported In Lieberthal (1512, 131b) and others. On postmortem 90 Kopon's Sarcoma 52 hapo lexon spuiting stars ecco 53 alicular hemorrhagl dusculoration of the foot. examination, Saphiet (219) found enlarged and inklurated vedou stems and netve trunka. Hilstologically along and within the vessel walls and sheaths of pric trunks, there wou true typical of Laposi's sarcoma Brand and Seuffer (29) observed the development of such a tumor from its origin from the wall of the used to its attachment to the skin within 91 houn The patient reported by Webstet (971) displayed a prom- Ident vedows network on the palmar surfaces of the left hand and charged veins of the right forearn He believed it um Symptoms ol Come on to find anall, karlized srous of telangiectaste on the trunts, which liber derobped foto typical Laporte Arcima lesions. In addition to the typical wulancow lesions this pas tot bad deep-seated, blush green, branching streabs on the legs, which, to places, wklestad out in the manner of varicaso veins and were for and palpable through the skin. Histobogically the imallest of these streets are found to be on SHOWU veuse without two paying chwager. Howeter those of a danctor larger than 2 to 3 mane sboured aggregations of spindle cells closely paded about many mal blood pace, with cotx pigurent deposited to the region. When these re glors were followed to serial secttoos, it was found that the blood spuses beste confuent and terminated in a thin walled vein, without spille e muede, at the point at which the treak narrowed. In light such fact, Weert believes that the venou network on the left hand was a proromal manifestation presaging the appearance there, at alle date of typical cutancow lesions, had the patient livet Lack of Pigmentation. The patient described by Wolf (287) was a 46 yer old man who had an emption of min. ) cars duration on the feet by, thighs, trunk aral uppy cutremitiem. Tere was diffuse blufth-red inaltration on the outer and lone sde of both fers, and several bluish gaduur lastoas on the back of the right bace. These rere alight cleration which trere firo on palpation the truth and upper etteities. These shocolored lesions produelty developed into typu bluishred soduker, Bocording to Peck (193) Pollzet (1990) commented that Kapoor's orighal Dame tos klopathie mul aple pigmented to best pigmentatiop us about to this patient Pautrum and Dar (1916) Lescribed a patient whose lesions becan use colockas, painful deep-seelod nolules, de tched only be palpation, which late asumed the character bile oltaruu true Tropiic Chances The 10 scurok then, ranted by Cistella (1.1) bla cutin our stuption of seven cant duration which companied by edena of the left ouer outroqitu Ils 83 Kaport, Sarcoma also had leukonychta involving the distal two-thirds of tho finger and too nails. One of Sympicka (SL caro 8) patients hd dubbing of the ftogers Verrucous Diderd add] Hedman (55) and Peutrier and Diss (101b) called attention to lesions of a vetrucow chur acter In a discussion of Schiller ct al (221) Finnerud (73b) stated that an almost constant finding and one not wually Doticed, I tho party character of the skin of the sides of the dorsum of the toes. Bloom (LJD) In a discussion of Arche (6b) called attention to the verrucous trupHop on the corsa of the toes, which may appear before any typical lesions of Kaposf's sarcoma Wises (2851) patient had a raised globular vermucous ledon tho stre of a small pieces on the left deel which had been present for three months and resembla a basel ccll cpitha liama There were arciform, naised, mooth, purpllah Infil. trated plaques and carly a perildal lesions on both palms and finger.. Similar various sized, iniltrated plaque, raised and superficial, were on the outer and indet borders of the role or the left foot and on all the toes. There was a palm sized, superficial, infiltrate plaque on the outer surface of the Night bech, with smilar plaques on the inner aspects of the solo al on tho first and stand toen, and thrco isolatcal, pea staat, pupish Lestons on the aptarlor aspects of the left leg. The left foot and ankle were edematous Small lymph cocks were palpuble in the anterior cervical and exillary nglons. Bullow Other unwil cutaneou features consist of glistet- Ing soulevhat translucent podules which resemble thick walled vesicles, bullac and lymphatic custs such as are hen in lymphengloma circonstiptum, according to Vacker anxi Cipolleso (155) These are the stee of epea or a cherry and may be punkish, brownish or bluiste to color Such lcslong havo beco described by Plc (1955) Ormsbr (166) Frost (83) Dillard and Wetumon (53) and Wise and Eller (2850) hlabt(1335 ) patient had tonsite sheets of deep lilac colored celematou infiltration, shich was falsh depressed on both hands and fect. On the forums and legs HCTO Symptomt 83 DUDcTon decrete round a ona lesions, of similar character On both enlla there were marledly dematous arcus which wuro abnost bulous in character A few nodules had appernd on the right buttock within the past few months A patient with edemns and bollox laston on the legs weus proscatol by Plothman and Henningsen (213) It is also noted that in kupori's sarcome, especially on the lower extremities, there are occadonally soft, reddish brown Dodules which cau bo prabod foto the skin, kell below its level and which gradwally reappear in the course of a fow seconds, bo resume their puffy appearance. This soft, almost cystic consistency of the berdloos is probably doo to hmoph susta. This feature was also noted by Roochese and Kero (100) la to patients 73 year old Italian women who had a bleeding tumor of the left lodox floger was reported by Torry (258) Both hands wero diffusely cyanotis, with patches of deep purple color On tho skles of tho Angers, trero regularly shaped areas of infiltre ton como flat, other por sized Another pea dzed area of Inftration involved tho Hght great too and thero wero per plush red lat, blotch-liko in Altrations on the midlo of tho role. Abstologically the finger losion confirmed the diagnode of Kaposi's sarcoma. In the discussion, Morron (173) called attention to the large rakles ta tho webs of the fingers, Ho believed these symptoms to be reso in Kepos's sarcoma, although they wet characteristically present in this patient Wie and Eller (2530) described bullous lesions in a 06 year old woman w bo descloped reddish brown, voleceous patches on the peck, hands and feet, 15 yeus provonly These lesions increased slot ly in size and in tho depth of color Four or five years after onset the decase was nearly general Luah loculing the face and, to a lesset eatent, the scalp. On the anterior and posterlor surface of the right thigh, thart Heto large roos of closed grouped voicles and bulae, both Isolated and fuxal Theso eskuabulous lesions aroso in Intrated and thickened areas of Ho. The bullee ruptured cally and spootaneously emitting o profuse norfeld, clear watoti discharge which caused the affected area to bo moist
91
Koposto Sarcoma
51 Trucow leuon of the toe.
55 Development of podle cell uncona in lesions of Lepod's
ULA
Symptoms 85 and uncomfortable. Whilla tho lesions acre pot typial of troo vesiclos ud bullae, they were ambogou to the valcular efflorescences of lymphangioma drcumscripture, so far as tho fuld content was concerned. They Aromes a result of lymph stesa cmed by constricting bands of kin above the alla which gave rise to an intense lymphodeme of the skin above to constricted greu This edema eventually caused the fama thoo of the veiculobnlou landoms. Histological semination rovcıled a characteristic anglometow sercome, the true a hibiting an anggual dogro of lymphedete. These lyoph cytt in Kaposi's arcoma have ako beco desobed by Della Prveta (52) and Pick (1884) On the other hand, Trimble (281) stated that bullao do pot occur in Kapod's sarcome The leximere lymph vedlckes rather than bulle, which are formed in connection with the lymph stans. Akwe, et oL (4) described a 53 your old Greek man who doveloped a beun dzed vesiclo which contained darl Aud. Odo week later the ball of the foot beoemo painful And blulih discoloration appeared on the dorsum of the foot. Eloven years later the histologic diagnosis of Kaposi's sarcoma was madlo. Sio years later binlab papules appeared on the Lowrer part of the left leg us pelu on the bandı Rostenberg (219) prezented a 58 year old man who had developed lestar four years proriously On the role of the left foot there were a number of isolated reddish to blush maculas, from 3 to 6 cms to diameter which appeared to be walls of the bullio on pressure. One of them contained a clear Aukl. Theo Ja band liko ver underneath all the tous, about 8 cm. wide, which had the une reddish appearance as the analler lesions and also appeared to be the wall of a Love continuous bulla. On the dorsal face there we prominent lescoo the size of a large pez, on tho cond tos, which od pressure also appeared to be below. The first and second toes were considerably cnlarged and the bony struc tures were ako imalied A 39 year old man reported by Cougetot, et d (02c) had polimorphigo of the lastoas associatal with bullie general Kaposi's Sarcoma tred arthralgia and erythematous stomatitis He had do veloped successive and continuous crops of purpurlo papules on all four atremites during the preocding four months. Thcso lesion developed in tiro or three days time then dis appovod or becamo DocTotic and encrusted. There were also several bulloc on the thigh and large thickened red plaques on the back of both wrists. 7 Ecolution of Eruption. As a rulo, tho crupulon evolves Howly and the effection 4 progressive according to Jlach.co and Cipollaro (155b) Od lesions often undergo spontaneous involution, Leaving atrophy depressed scary and pigmentation. At the samo time, now nodules or plaques appear Occasionally grouping of lesions, and especially the developmont of nodules at the periphery of okler lesions, produce e con guru than suggestive of syphilis. Tho tumors grow slowh and after a variable period of timo sproud over the skin and Involvo tho viscera, particularly the lungs and the lymph nodes. Hemorrhages from tho cutanoow losions and Vlatti frequent ly occur and there may be edema of the leg or the foot and elephantiass-like changes Tho rate of progresdon is slow and variable spootaneous remisdom and regression of the stons sometimes occurs, leaving pigmental atrophic scars. The dura Hon of Ho averages between five and 10 year death being due to intercurrent infection, cacheda, viscerul involvement or repeated hemorrhages. Androws (@b) reported a 50 year old man who developed a purple plaque on the medial side of his left anklo besen years before. During the last threo vean purple plaques and Dodules, verydog from dime to givet dollar ste developed on each log and dmilar indurata plaques appeared on the night uhle The tocs assumed a frostbitten Appearaner which had perrsted. During the past two or three years the trunk had become involved, and in the past year the upper morem itics and the fact There are frequently variations in the classical macule podulo and plaque formation according to VcCarthy and Pack (153) One type adhes marla fibrous reaction and clinicalls Symptomer 97 Dembla nourofibroma. In one of their cuss tho disease was rather to dolent and remained localized on tho Lg for 10 year Ow lesions will often regreta into a flat pigmentod ser in the periphery of which bew modules may develop. Still another typo is composed of pink transluecut sodales diallar to lymph englome Lymphadenopathy is common in all phases of the di case, and the rodes may or may not cootain actual spindlo celle Ulumately the timon Day appear to the rubmucon of the entiro gastrointestinal tract and lungs us friable, hemor thegls potypold martes. This development Darks the trand ton Into a terminal pbase when death fruta crenguimtion L Imminout. Not uncommonly the tanuls, thoracic aux abdom Iinal rodos ond booo are lavaded by the topor Three of thetr publent (153) haul tavohraient of the oral cautty four had lavalvesent of the lungs, two had bone implierneut and ano bad imoleucot of the lher and speed. Davlations from the typial cutadoon onset hva beca ro ported and the tuman mey originate in practically any part of the body However phor to Dooffefs (56) mooograph io 102 it was generally beliened thut all airculmeans lostons WCTC metastatic, Inaanuch as up to that time fev quo had been described la which atacou kolom werd absent or had been precrued by visceral lesion. Since the Keral patkats harving tumors in the veces, Bot associated with cutaneous manufcrtattoos, bare bern described. Primary Kaport's rar come in the heart was decorte) by Aegerter and Peale (2) und Chorsset and Rappen (40) in the bidder, liver and tnter tincs by Teleschi, ct al (957) In the lymph nodes and Tucou mera hrane of the peo's by Burridget and Deen (13) and in the melict by Grahain (3) Toks erhalta prests tbut Kaposi's woma may be a systemie discute o mulucenthe origin ood long duration with Bilo tendency to pretastaula Sequeira al Brain (3) belford that these C16the powth appran to underpo tetropedic changes with formation of depigm oted depressions Twe are quieuit of the spindle cell WOOD# la o petkn! *b haposto arcoma a reported by Sequeira anal Brain 0:9 Kapod's Sarcoma (2330) This noen doveloped a purplish discoloration of the sidn jut above the left ankle at tho ago of 41 year. Tho con ditton spread slowly and after di months time completely covered the dorsal face of the left foot. The following year lesions appeared on the dght foot and slowh increased in die a year later similar lesions appeared on the dorsum of the left hand and wrist These letter lesions were pour to and tho feet becamo painful and edetabout the dorsal surfaco of the left foot ww red and swollen, with w area of raised purplo mal tration ACTOS the matep the lower Umit of which was an almost straight Hoe between tho malleol. On the inner side, thl line was made up of small discreto popules Tho toos were almost Immobilted by a sclerodermatous condition of tho sn covering than. About 5 CDs. above the Internal malleolus was a discrete, roughly squaro-shaped ledon, 1.5 ans ACTOSI, consisting of a raised purple flat topped nodule, On cumination 13 years later the skin of both fect, and a tending 2 inches above the onlles, was hard and thickened and produced some limitation of motion There were numct ous, scattered, purplish-red, soft, slightly raad nodulot of varying sizes over tho upper part of the lego A fongating man, arising from one of the outlying vasculartzed noduks, theo appeared on the front of the shin and the leg was amputated The patient desobed by Formad (79c) developed an ular attoo involving the inner aspect of the left ankle and foot 99 years after the onset of posts sarcoma listopathological examination disclosed a probablo prickle cell thelioma. Gross (101b) reported an 81 year old man who presented soittal bluish red, some hat in illtrated patches of firo con sistency on both ankles The notace of these Iestons was slightly scaly and hypertrophic. There iras moderate olema of both feet and slight vunucus hyperlitatosis on the dorsal surface of the tous. There around, dark red lolxLatoul tumor with a narron bave the size of a largo marble on the outer espect of tho left foot The surface was partly deuded ul cared with a grestish bloody crudate The luftological Symptoms 99 camination of the fungating humor showed changes of low grade pindle cell wercome Satenstein (2205) stated that many cases of Kaposi s sarcoma froquently have fungatlog tumors. This was a comparatively common Anding, when Injury to a tumor occurred and destroyed the skin covering cuing the tumor to grow outward rather than towerd, thus resulting in a fungating lesson. C. llanorum In the majority of cases tho hemogram to normal. Anemia La present in many cases, according Dorffel (50) who reported OTC patient with a bemoglobin of 33 pet coat, others with varying degrou of anania and some with normal hemoglobin values. Similarly the red blood cells numbered from two and ono-belf pillon PCT al mi to normal. VacKeo and Cipollaro (1556) state that secondary ademla is quite frequent and may bo severo The skin color of patients having marked anemia w. described a day or lemon colored by Bernhardt (19) Do Amics (53) Riehl (207) and others. The actutance of ctnla has also been reported by Stelna (247) Hartzell (111) Funk (81) Variani (160) Lieberthal (1511) and Dhard arxl Wellman (55) There la do characteristic total white blood cell count, as rescaled in a study of casos in the Utematwo by Dorliel (56) although a few patients had definite leukocytosis There WCTO fev abnormalities in the differential whito blood cell count. There *ocrasional disturburo in the relative proportions of polymorphonuclears add lymphocytes, but these were uan) ly tot dgnificant The most striking feature wu the idet oble degne of monocytosis. Two patients with an inacuo of reticubenlothelial cells and monocyta was reported by Bet trectal (210) lp these cases, and in to other he found concomitant centations from the normal letnogram of the periplural blool malals a marked monoa toss Flaret (77) desetbed 20 per cent abdormal pronocytes in the peripheral blood with a marked shift torud cells of the hmpho-moro- 100 Keport's Sørconia cytic sene Wendo (276) reported 1212 per cent largo Donocytas, Dalle Favera (59) 282 per cent large monocytes and 8.0 per cent transitional cells, Gegorias (93) 25 p cent transitional cells and 6 per cent podnophil, Rottonan (214) 5.5 per cent monocyta, Wintcrnitz (282) 8 per cent mcmocytes and 8 per cent eosdrophils, Paolini (189) 8 per cent monocyta, 3 per cent eosinophils, 2 per cent Turcko cells, Bechet (14) 9 per cont monocytes 8 per cent codinophil I per cent transitional cells, and Vigue (2002) 0 per cent monocytes and 3.5 per cent cosipophils. Dartfel (58) found eosinophilla to be less frequent than monocytosis. Hurlbut and Lincoln (121) also found that cosinophile is not a constant fading, although 4 or 5 per cent cosinophils to a normal total leukocy to count ius not uncommon, and occasionally counts up to 17 per cent weru rocorded. They did not observe monocytors. Ells (61) reported 46 per cent lymphocytes and Awczow (122) 55 per cent lymphocytes in tho penpheral blood Dupont (586) reviewed the blood coumt cluange lo kaposis sarcoma, in which the peripheral blood wu compatiblo with a kukemic ryndrome and described thrco additional cuscs UncomidoD types of cells were found, which wero clasified according to two groups 1) an uncommon type of lymphocyte, and 2) more or less atypical largo mononuclear. T1xc Arst type hu a kule shapel, bilobate mucleia, either doublo or having one or tivo mall bud shaped cipandons Changes in the sternal bone marrow comparable to those present in the spleen hete occasionally obscrual by Tedeschi ct al (257) Active hyperplastic bone marrow was found in two cases by Stats (246) although thero ut do change in the peripheral blood picture The changes were due to part to radiotherapy of the sklo or splenic lesions Jis believes alterations in the hernogram are rarely marked anel neer of specific nature Hemolytic anemia has been reported in association with Lapost's crcome. This type of anemia is a fairly common finding in the lumpboras. The che described by Gretti od Symptomt 101 Bottoni (87) 4 unique in that this patient had a micropherocytic bemolyto ademla with an enormous excretion of feal urobillogen. In addition there was marked granulocytopenie and the patient dial with oral reports. The cause of Uskcgllo, ct al (281) may have been dollar A moderately SOVETE anetla i leukopeole were temporarily umeliorated by splenectomy but there was no definite evidence of a bemo hytic process. Two of Stats (2446 cases 1 and 2) patients had an active or hyperplastic sternal bone marrow with a normal difcrential count Bertaccini (210) Ciamined the bone mar row of there patients with Kapods sarcoma, all of whom had considerablo modotosis in the peripheral blood. Two patients showed an incraso in reticuloendothelial anal modo cytic cells in the martov Impresiog smear from Laposi's tumor Dodules revealed monocytold cells similar to those found in the blood in certain causes of robacuto bacterial endocardius. Slartensson and Hearichron (161) described the clinical cours and pathological Andings of immuno-bemolytic anemia in a 63 year old man. He first developed what appoered to be llopathic aneinla, but one year later Kapadi sarcoma wu Magnowd at autops when anglosarcomatou changes were found to the hmph nodes, stomach, cothre lotatinal tract and very small lesions in one lung. Txere were no cutaneous mant festation. Spkocctomy had no effect on the bemolytic process which did respond favorably to treatment with steroids These compounds dll not homenat Inhibit the grourth of the lymph nodes although they regressed readih with rocnatgeo ray irradi tuon, and a quiescent stage resulted, but soon other change Ortured. They bellene that anania of tmmuno-hemolytic type may otur in Keposts surcoma es in other diseases of the reticulorndothelial system, and this ause shows that hemolitic anco mul appear at a ICT curly stage of tho discuse with minor pathologic cunges They believe this to be the thin case of bemolitie ademia in assoclation with Laposſ i sarcoma to be reported the first case a described by Creppi and Betton (gelo a patient who ba superficial changes of the 102 Kaposi's Sarcoma genitalia. The other petient, reported by Hogenban (120) was a middle aged men who had typical Laposi's sarcome of the skin, lymph nodes and internal organs in addition to a sette bemolytic wand. The whito blood cell and thrombocito counts were also loir The leukocytosis and thrombog topcola utte controlled by splenectomy but the hemolyds persisted. Tho significance of these varlou splenis, bemic and bono marrow destations cannot be assessed, according to Stats (216) There is a variability of findings. Somo reports pupport the theory of marked reticuloendothelial system involve mcot. IIc believes the changes may be interprete cíthet is a reaction of this tissue to the presence of the tumor or a direct Implication of this issue to the process of peoplasia. Dorff (58) has condseh presented some of the entdence to support of this thesis. D Internal Organ Involi cucot Although the skis is the primary organ Loveled to Lapsis sarcome, and many cases show no spread beyond the cutaneous site at autopat it is not uncominon to find surcomatous de posius to the vixera. Involvement of Dearly ceni organ of the body hus been recorded. Of more than 600 cases de scribed from 1872 to 1919 Mitchell and Fedet (172) found the common sites of Losolement, acluding the shen, to be the penis, gastrointestinal tract, upper and lower respiraton tracts, and the supcrficial and decp hmph Boiler The tmcommon sites utte described us the Stou menbranca, spleen, Hver adrenal glands, myocardit, mesenten Laphragm urinar bladder thyroud and brain In his series bortfel (58) found the skin to be most fre quently involved and the pott most frequcot site of imohe ment was the gastrointestinal tract from the mouth to the sectum. Sett in frequecks were the thiet luogs, retroper toneal and presentate hytoph nodes Lots frequenth involved were the splees. posteas, Lidocys, suprarenal glande perd toneum (including the omentu) testes, epididimis, trached, Symptoms 103 bronchl, pleure and heart muredes. Organ rerely involved were the central nervous system and the polpheral nerves. Choler and Romscy (40) listed the involvement of vixeral lalons, recorded prior to 1932, u occurring most frequently In all portion of the gastrointestinal tract, llver lungs, lymph pode (retroperitoneal md neentalo) froquently in the epulldymi, uncha, bronchi, splecaspancreas, lidocyı parl toneum, testes, and rarely in the nervous sytten peripheral perves, tongue, bladder coucles and patultury gland. Thoy Ilsted the brale, thyros, Orary and stores as nover lavlag been fnvolved. Hovrover curea of brato and thyroid fuvolvo ment have been described by Nesbitt, of oh (178) and Schitzen md Bulhardt (24) Kapods wercome not boclated with cutaneow Leeloos wu Ant described by Dorffel (50) A petient described by Greppi and Bettoni (97) developed tho pomery london on the glans penis, tho loguinal lymph nodes became involved and, at autopy thero ww o tension toto the peod muscle and nodulaa wero found in the lumg Pearco and Vallets (193) patient deurloped a nodule on his guta sveral months before the akin became involved. Two patients witb primary londons on the glans peru WCTO Doted by Barringer and Dean (13) Casco lo which the inguinal lymph nodes were the puuary Alte of the ducato were reported by Van Clevo and Hellwig (203) and Goldschlag (80) In the latter cuno, the original lymp sode involved was diagnosed as lymphogranuloma ingutule but he believed it to be 40 unge rohnlichen vorstadium of Lapod's sarcona Io e miso described by Stats (246) about 14 mootha Intervened between the one of the uhren, characterized by ymmetrie edema of both Lowo a tremities, and the apreuence of circumenbed coduks on both legs. It has been known for many years that the veta od hmph nodes may become imoked by a dollar Arcomatoua procru, es motioned in Dorite (58) Chotos.com and Ramsey (10) Vacher and apollaro (1556) Dalla Faveta (2) ao Lara (100) Because of this, the disease of general dg 101 Kapod's Sarcoma nificance and must be considered to the diffcrential dagnosis of a largo group of systemic complaints and physical fording In the first cases of this type observed, tho visceral involve ment followed the appearance of the cutan-cous disease. Voro recently Stats (216) Vackeo and Cipollaro (1555) Aegerter and Pcale (2) Bilandoni (22) and Vweglio, et al (281) have noted that involvement of tho internal organs may precede the appearance of Lapood's sarcoma of the skin, and song, as Acgcrtct and Peale maintain that tho cutaneous lesions ney be lacking altogether Horever Stats points out, iany cases of Lapost sarcoma of tho sklo progress slowly for many years and have been examined at autopsy without any indication of sproud beyond the dermal apparuhiis. Vany observers Dow believe that such fod aro primary that there is no metastus. Wito the first manifestations of the affection aro ordinarlhin the sun, there is ovidenco that other parts became involved carly or simultaneously and even that tho discese may begin in the visceru. Tho patient reported by Cholster and Ramsey (10) lud no cutancow involvement and the lesions were primary in tho auricle of the heart. This patient, a 28 year old man, was acutely ill There was dyspoca, moderate cyanosis, and the great vessels of the pack were distendel. The heart was marhadlı enlarged but there was no evident pubation in the precordial or great vessel cal Souls were distant ud without murmur The nmptoms became rapidly moro severe and he died sit days latcy The anatomical diagnosis wu hemorlagle Dooplaan of the heart, with secondar nodula in the pericardium, mediastinum, pleura and liver right cano thorer hemorrhagic Infarcts of the kidneys and advanced passlie congestion of the liver Incidental involsement of the heat and pericardial us, geo cally without clinical symptoms, ts frequently recorded among the visceral localizations, and is desobed by Symmets (954) Dillard and healmon (55) and Stats (210) Apr mary and unique curljac localization in more musul Almost simultaneoush in tho independent papers, Hellet (273) and Symptoms 100 Choissez and Ramsey (40) decided to co of primary Lapods Arcore of the Hight atdum of the heart, classified u angloreticuloexothebome from the morphologdo standpoint Horect Stato fuspects thao two report to be the same patient. Stmilar cases have beco desenbed by Culme Viotta (50) od Aegater and Paulo (2) have added another cutofn which the only apparent localization of the dont was in the Hght etrum of the heart. The identication of there can us In two of Kaposi's mercoms was based on the close ro kepblance of the cardiac finding to those usually ren in Asroclation with the cutaneous manifestations of Kapool's war comt. Vbether or not the camera dexetve to be considade ruch Is A Portinent point. Paolinis (180) patient complained of Intestinal symptoms before the appearance of cutaneous Lexicon. At dasth, the trual cutaneous manifestations and Dodule of Kapodi we com wwe found in the large and smal totestines and other vbscere. Involvement of organ puch as the intestines, hung and bloddet may be indicated by hemorrhage, cough diarrhea, cte but definite clinical indiation of involvement of other organ iure likely to bo absent, Igpored or mwinterpreted. There is often no certainty of borral partidpation til DOCTOpry Phulpmon (197) Dalla Faveta (52) and Hanson (103) described the fineling of multiple cutaneous lalons without bertal involvement at autop) There WeTO DO traced of viaceral metastast in Hansson 1 patient, despite the fact that the disease had been present for five itens Occudanalh ap ante mortem diagnosis of verceral favolo mont prior to the appearance of autancow lesions is made The me shunga occur in the viscera 21 in the skin. Thus fact, together with simultaneous in oh coknt of the skin and keeru point to internal lessons as betog of primary and in dependent origin, and not the metastads, according to Becher and Thatcher (156) True metastas's docs Occur atat ma- Uprunt ilyentation, but many case reports or indefinite oo this point 100 Kaposi's Sarcoma 1 Lymph oder A mare or less geletalized hmphadenopathy of limited atent or indefinito character may affect both the superficial and deep hmph nodes before tho appear Ange of cutaneous lesiont This lymphadenopathy might cash bo overlooked or misinterpreted, according to Tedescht, et al (257) Houta lymphadenopathy does not necessarily in dicate that the lymph nodes are involved in the proces. Then belice that it is dificult, at present, to discw the signißcance of these changes Ode possibility is that they may represent a Don specific inflammatory reaction, the other that they may represent early stages of development of the disease proces. Hyperplasia of the reticulom cells was believed br Dorffel (58) to account for this condition. He states that hypost's original conception was that the lymph node in oh count oc curred and when the serconnatou leglans were ukeratcu or secondarth infected Subsequent obseriets held this summe opinion. He described cash insohement of the hmph nodes us characterized bi swelling and proliferation of endothelial cels lo the blood vessels and in the supporting retkulauta dothelial tissues of the Dades. In some cases there was no histological evidence of tumor tissue DAL Fastra (52) was the first to call atteption to the fact that the colarged Dolles may dot sbon typical focal changes. In his case the only detectable change was thicken ing of the brous connectic tissue stroma of the lizoph nodes. Greppi and Bettoni (97) described round cell hiperplada and Cokischlag (90) found a cluonle nonsped6c inflammatory proces. There as a sarcoid like picture in the cause described by Vise (2551) The cases reported by Vacher and Apot laro (155b) showed plasma cell infiltration histiocytic plega tes containing frod bioment, and moderate thickning af the stroma of the lymph nodes. Cars without histological afdence of tumor tissue wete also described by Stats ( 216 cure I) and bi Dillard and I calman (53) Frequenth the hmph boles are palpable and the swelling mas be due cottrely to inflammation is described bs Hartzell (111) and Strattoo (052) At times the carls lesions are smaller and Symptoms 107 there is proliferation of the endothelial colls in the blood vor els and rapporthog reticuloendothelial ytan of the poco and soveral cures in which the lymph nodo histology was typical of Liposi percuma have been docubed. Among those describing these featurd wato Hartzell (111) Krupnikow (110) Verland (180) Selled (230) Stratton (252) and Wise (2855) Although it is not possible, at present, to 2xco thoso Andage, they may repesent the epily Auger of lymph podo alteration before tho tumor appears or may represent secondary inflammatory change, coording to Stabs (210) On the other hand, Beringer and Dean (13) ore of tho opinion that the lymphatic nodes are not involved in this dlaces. Stats (246 Caso +) observed that, in early lymph nodo lo volvonent, the tumor appeared first in the pertrascular areas within the nodes. Often, ruch nodes wero entirely surroundod by Kepor tumor which did not imdo tho apsula Lymph nodle tnvolvement is frequently wo marled as to be out of all proportion to other visceral Invohement by tunnor This may bo coaxtrued as evidence favoring the general systemic daturo of Kapool's sarcoma and in this regard relates the discuso with Tymphoblustomes or reticuloendothelioak, bo believes. The deep lymph nodes are more often enlarge than tho super Adal and a wtuation which makes clinical detection difficult. The presenteric and retroperitoneal rodes are usually involved although all or any groups of nodes may be affected. The hymph polo Involvement is not necessarily regionul to an organ Imolyod with leposi's tumor and therefore, this molvanent should not be regarded us a metastatic procent Enlargement of decp Dades can occasionally bo ascertained clinically by palpation of imegular masses in the abdomen or by rocnt porologle or clinical aidetice of mediastinal or bronchial lymphadenopathy Histologically the appearance of the losolie hmph nodes may be identical with the cutaneous tumors according to Stats Afarled enlargement and matting of the hypogastric perzor 108 hapod's Sarconia tie, tracheobronchial, paratrachal nedlastinal and mesen toric lymph nodes wero voted by Stats (+6 caso 9) Ou section, the nodes were fleshy or hand and grey to bluish browo to color and histologically were pantally or completely replaced by anglosarcomatous tone. The cervical lymph nodes cnlarged progressively in another patient (caso 3) and at the time of death there was tho aspect of a bull pech Another patient (2H CHO 4) had marlad enlargement of the tracheobronchital, paratracheal and paraortic lymph nodes which were matted and adherent to adjacent structures. Thero wu axtension of tumor Hissue Into and around the Aprarenal glunds (tho left was largely replaced by tumor) piacTcUS and Widaoys, while the same proces could be traced close the lloc versch. Tho mesenteric hmph nodes were onlargach feshy and bemorrhagls, but not fused The Veixent most severe toward the root of the mesentery Inguinal lymph nodo tavoliement was reported by Gokl schlag (90) left subma vllary lymph nodo involvement by Arauz, et al (7) and by Grepp: and Bettoni (96) TheTO lymph node involvement in one case described by Victtech (109) and involvement of the mesenterks, arvienl and gas tro-hepatic lymph nodes in the case described by Dinard and Weldman (55) In Symmets (25-la aue 8) cuse there wero palpable nodes in the left ouille, both groins and borb popliteal spaces Bechets (14) patient shon cd mashed inguinal, anical and allan hmphadenopathy Gastrointestinal Tract Incolremont The gastrointestinal tract I recond to the skin as the most freixent viscrtal kcal zation AN portions of the tract, from the mouth to the ani, may be involved. The oral area, stomach and soul] Intestinas are most often affected the large Intestine and appendix lest frequently The oral ninifotations are described unde ETIOLOGY (Sites of Predllection) The gastrointestinal symptoms are usually not pornident but paticuts having diartinez, berontage en henonlagic eludop loto o setows uac have been desenbid heyertet am] Peale (2) reported sitte carrber alxInmiool pain and Symprome 109 bemorrhago and Paolini's (189) patient had severo derrhea and abdominal pain. Stats (246) states that literally hundreds of Dodular may be suttered throughout all seyneats af the pastrointestinal tract. Pharynk. Bilenconi (22) dentibod 1 62 your old man whoso vodco bed recently becamno guttaral and neul, he had difficulty la salowing and a sensation of obstruction lo the throat. Theo ku a mobile, rounded tumor in the hypopharynx bot no lymphadenopathy He also had DUNTOWS tumor, some ukeratel, on the hands and fect. The histo- Logical diagnosis was Kaposs sorcome. Tracheotomy was paformal and tho hypopharyngeal growth removed. This lesion was found to have the same histologic strukture as tho cutmeon Icsions Involvtment of tho phary nr was also reported by Schirmunskaja and Tschouchia (223) and bry Sym- DXCT (254) A patient with lesions in tho pharynx, docribed by Arutz, et al. (7) had a tracheotany add the buccopharytgeal lesions wito treated by clectrocoagulation Stomach and Intcrtinet. In tho presenco of gastrointestinal insolement, the mall intestine is more frequently moveu than tho stomach. Stats (216 caso 1) described patient who had, throughout tho gastrointestinal tract, in the greater curva turt and fundu of the stomach, doodnum, Jelumut, Deum, aprend, anal colon, purplish, drcumscribech submucosal Dodules, up to 15 cm ti diameter profecting from both the mucaul and stoval prices. At the point where the cop agos passed through the diaphragm, there was a codicoution of tumor ter which infiltrated the wall to the mucosal Aulace In another patient (246 case 9) there was a flat Dodiche 3 mms in dancter in the submucosa of the greater cun ature of the stornach A few similar nodokes 4 ETC present to the presentery and in te small intesunc at the attached bordky Shining through the serosa of the free portion of the hull intestines end the large intestines were many pea stacd humor noduks. There were all situated in the mucosa and submorosd und weTE IM Ted with otact epithellum. In DTTS (112) patient the stomach ku dilated and
110
Kapod's Sarcoma
58 Hatologic action of toogue sbor og ako or subepithelial
contacthe taste and diffuse probferution of capelines. (Couto
of Samuel Zulon VID)
57 lavolicment of new and equoram (Courtesy of Sumxl J
Zlon VD)
Symptoms 111 on the mucous membrano along the greater curvature, wero many groups of dark blue, superfidal, confloent modules. Tbaro were two dmilar podules in the duodenal mucous mem brane. Ono nodule to the large intestino was located at the place of attachment of the mosentery and tho others nearby Zalans (250b) patient had lesions on the vermilion borden of tho lower lip and tongue. Ho (284c) reported the autopsy finding three months later The entiro gutrointestinal tract, cacepting the colon, wu found to be studded with lesions of Lepost'. Arcome. In spito of tho fact that almost the entire Iwonen wus occaed, the patient had dover complained of gastrointestinal sy optome. Barlcon studies of tho gastrointestinal tract of Seagraves (228) patient revealed numerous intraluminal masses of vary tog slzce, closely chustered together in the mall intestino. TheTo was evidence of low grade obstruction Surgical a amination revealed the entire small intestino to be involvod. Pathologlal memimallon of 14 fect of the resected anall Intextine, showed large, blackish red, poły pold masses, ranging from 0.5 to 80 om in diameter projecting into the lumen of the bowel TITO were H gross lasloos counted in this spectrcm, 23 of which were concentrated within 18 inches of bowel Tho lesions were trregular moderately soft bad a fungating appeamnce and some weto undergoing involution. In addition to the polypold masses, there were numerous purplo red povuks lying completely beneath tntact mucosa and ringing up to one om, in diameter A 17 year old boy who died of intotineption awd by a soltary Kaposi nodule to the Lleum, fu recorded by Tedeschi, eta (257) Involvement of the gastrointestinal tract was do scribed In Acgerter and Peale (2. caso 3) who state that the most common awe of death in Kapoor's sarcoma te im otvo meat of the intestinal mucos with ulceration causing hemor rhuge and edugulation. 109 year old man who had ipvoliment of the lejunum with perforation and peritonitis, was reported by Vitchell u Fede (172) le had cutancows Lovdhement for many cars prior to the perforation of the
112
Kaposi's Sarcoma
58. Vuone of theton repliced by a diffuse drcumscribed inAltra
oma (Courtesy of Suru y Zaloa, VID)
K9 Jasolscent of the Iner (Coutcry of Samoch J Zaloo, ID)
SyAPTORU 113 secondary mess in tho lefunul. They bellove this to be tho Ant report of perforation va complication followed by peritonitis and death in Kaposi's aromu. Lavolvement of the gastrointestinal tract has also been described by Cholster and Ramsey (40) und Heller (575) Darife (50 cuto 13) Van Clove and Hellwig (985) and by Dillard wul Weichnan (53) Lleer Iorolvement of the lira hu been reportod by Van Cloro rod Hallurg (28) Dottel (50 caro 13) and by Aeger ter and Peulo (3. cure 3) 3. Respiratory Tract Nearly all portions of the respiratory tract, with the aception of the naml avity have been involved by tumor pocula of Xapoor's pucana In ono of Stats (218 case 1) patients, the plural surfaces of tho lungi treco studded with frregular Laut, Lemorrhagle plaquea which penetrated the pulmotry parenchyma lo roma placak. There inero porcrul discrete tudor nodules to the right uppe od left lower lobes. At the hahus of ouch lung thero was a marted condensation and increase in Erm, bemorrhaglo LAIO which lovaded both louer lobes The connecthe tacte of the modlastinun und rctraperitoneal arcus was largely roplaced by bemarhagic tumor tlore which bound the various structures to one another Twpor involyminent, u erteudva la this case, bedd not been previouły recorded and despito those widespread lesions, Stats states that symptoms related to mach tumor aro pot coramon. In almost all Instances tho nodules retained gnall mel circunctbed and did not worate A patient reported by Berker and Thatche (13b) prescola wriglont tous plaques to the perletal pleurs, consisting of blood allod spaces, lped and bordered in places, bv elongated moille celle Involent of the pulmonary purcochyma and pleura write described by Dalla Parera (52) Lang hulve TIt wo ubo descend hv Dandel /50 coco 13) od Greppi and Betton (T) Lesuns of the lungs btre found at autopay in Wheel (104) puient Duula Faith (52) also de wibed love in the benchi 1 15aar old man who had a calon at the untTHr of the Hight intricle of the Laryat 111 Kaposi's sarcoma was presented by Rothman and limoninguca (213) Involve ment of the Larynx was also described by Symmet (234) Bilancioni (22) and Schirmunskaja and Tschotrhla (223) The patient reported by kuczow (12) lad groups of bluish nodules on the mucox menbrano of the truchas, par ticularly on the anterior surface, which were confluent and protruded into the air pasage. Dalla Favera (52) also do sarlbod involvement of the trachen Comel (45) described two men, aged 68 und 74 years, respecthely who had lesions of Kaposi's arcoma for several years. Both patients complained of cough and hoarsedes on both had small nodules in the laryor and trochen wlich had the same histologic structure as the cutaneow noduks. Splem Incolcoment. Pathological altctations in the spleen a clinical aberrations in Ita function, have not beca described frequently in the generalized forins of hapost's wr com. This may be duc in part, to incompleto obkrvotior, according to Stau (216) who believes that cytoo splonomegaly may be present to this disease. The organ mas cutend anywheto from the costal margin down to the hypogastrium, to the right of the midline and may occupy tho tatiro left opper abalominal quadrant and considerablo seg ments of the left lower quadrant and epigastrius. Thero WETO unquestionable changes in the spleen in three of Stats four patients and possibly in tho fourth. Lotto cases (cua 1 and 2) the spleen was nodular and sized to the posterior parietes. In one patient (case 4) the organ was enlargru seritul times the normal size but was free of tumor The Valpighian follicles were sharply delimited. The reticulum cell hyperplava in the pulp was striking many of the cells Weto vacuolated or showed degenerative changes. In another patient (care 9) the spleca weighed 700 gms., tumor nodules HTTe not recognized groush but saino hemorrlage was present Vicroscopically howmet spindle twr cells were observa la pooth drumscribed blanis infitrating the surroundlag tissue.
Syniptome 115 Greppi and Bettond (97) reportał moderute sbriolege of tbo splecu followymg the sobautaneous infection of epdoephrine. Histologic cumination of material obtained by splenlc espin- Har rosaled 7 per cent noutrophilic polymorpboouclear loukocytos, 50 per cent maturo lymphocytes, o per oent hus- Hocytes and 98 per cent monocytes, many of which were bu mature. However Stats (216 case 1) noted no significant change in tho size of the splora and found on cotirely differ et histologle picture with more lymphocytos and few Wils Hocytos and monocytes The pathological appearance of the plece rorles gætly in different casa In Symmer (234) cape the spleen weighed 100 gmi and the pulp was almost completely re placed by circumscnbed tumor noduler Paohnt (189) do sorbalu u to which the spleen measured 90 ans in its longest diameter Tho poonal architecture was replaced by many young tunor nodules dissominated throughout tho or gom. There WW also myelold oxtopless and the demarcation betweca the pulp and Malpighlan corpucl ius pot distinct. In some cases of hapoal's mancome with splenomegaly diy tinct tumor touo could not be found in the spleeu or if present. His not respondble for the enlargement. Greppi and Bettoal (87) four arterial hypeserta, typical of the alter tions in hemah tie rates Dalla Favera ( EU 3) found the splen to be moderately enlarged but without evidence of Kopods partoon. In the case of Ulteglio, et al. (981) the spleco Helghed 13-40 gms and contemod many well drum koribed noclulas angitng of large phagocytas Aled with red blood cells but no tunor Hssue 9 found In Voors (160) patient, the splenu dekbed os folicular Τιτα podules were found to be responsible for splenomegaly in the care described by Ian Clevo and Hellwig (285) 5 Urogenital Trect Atencys in one of Stats (216 x 1) patient topor tsune lovacked the hidneys at the hillus, scrotupanytog Large tael, and a few that plaques wtre apparent toder the apoule In 116 Kaposi's Sarcoma volvement of the kidocys was abo dosenbed by Vlictrecht (109) and Van Clevo and Hellvig (285) Bladdet Involvement of the urinary bladder was described by Dillard and Weldman (55) A 76 year old man wbo had Lapodi's sarcoma for 17 years before the development of bunaturla, was described by Smith and Samitz (213) Cystoscopio examination revealed two yellowish sodules on tho left bladder wall Scrotun. The patient reported by Aegerter and Pealo (2) showed Imolvement of the scrotum. Penit. There was involitenent of the glans penis in the cases reported by Barringer and Dean (13) Greppi and Bettoni (87) Pearce and I alker (192) Roger and Vigno (208) Bluefarb and Webster (240) and Ronchese and KCTO (2106) (Sec Figs. 18, 17 and 18.) 6. Bone Inco comer Occasionally thc bony gutem may be Involved. Any part of the bone, incurling the spiphyds, diapbyss, Martow and perlostrum may bo affertal. The nxost frequent sites of bono involvement are the hunds and feet where the phalanges may be comploteh dostroved Lesions occurnog in the bone may be due to autochthonous origin of tho Kapoor's tumor or to direct catcasion from soft tissue Bone involvement was first described by Scholtz (238) in 1899 Ho found considerablo decaldication and much do struction of the skeleton of the foot in ono putkent. The foot wous amputated and subsequent histologic cumination proved that the bono wa kidied with sarcomatou tissue Aegerter and Peale ( case 2) reported a 63 year okl man who had developed a small nodule on the outer aspect of the left foot three years preriously A few months later a solar Doduk appeared posteriorly under the lateral malleolus which TC4 rapidly and apparently fused with the first muss. A similar codule appeared later under the clorsum of the prot imal phalanges of the third and fourth loc. Roentgezogramı showed a soft tissue tumor invading the boue Iistologie cumination tecated Kaposi's sarcoma and the foot was an putated Symptoms 117 The patient, desobed by Fet (70) Wu a 53 year old American man whose bono fnvolveunent ww preceded by lesions to the mouth. One year after omset a fer nodolos appoured on the togtop of tho dght foot. They were rather from warty end of a dark brown color emell olceration occurred following surgical removal of u lexon for histopathologic study This memination rovealed Keports Arcana. Tho ather foot appeared warty and thickened. Cefalio (31) pore sented . 20 year old woman who had cutaneow lessons of Lapodi's eroona in addition to ostcoatrophy of the bones of one foot A patient who had cyst bko arons of rarefaction in the bones of the hand and foot wu descnbed by Roncheto and Korn (210) A 61 your old man with visceral and cut taneou lealous of Kaport's marcame wus descubed by Sherwin and Gordimer (230) Roentgenogramy of the right foot and lek which were the press of cutuncu tuin.OT, showed con- Aderable demineralization of the bones with what appared to bo destructive process of tho os calcts. The leg was amputated and a longitudinal section disclosed a fugating growth catmading from the skin, through the muscles, and into the as calci There be report of a patient who doveloped Kapoor's For cond at the age of five years who also had otporn chunga in the left pubtc bone left fltum und berus (Wbloslapp Vul, and Central States Dormatological Association: 37) Nesbitt, et al (178) reported a case of diareninated veceral Lesions of Kapods sarcoma without cubapoous lesions. At autopsy tumors were found in the thorecte and lumbar verto breo IIistological camination of the bone lostoru revealed a granulomatous hemoothade parcona with ploomorphic splodle cell u tho domtarnt structural anſt. Roentgen me amination of Kudelow. (IL) patient showed definite atrophy of the bone substance within the third to Afth lamber vertebrae Lateral especially on the fouth lumbar vertebn, Weto periostoal sodesin puts, nareduction to the third lanber icrtchra nurowing of the Intervertebral spec be tween the fourth and fifth lumbar vertebrae and an idio
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60 Cynthe dress of rarea to (ourte Frukex
Roncbre AVA Arch. Derou (Syph 031-1931)
Symptoms 119 tinctueu betureen the third and fourth lumbar vatebrna Frolich (82) presented e H year old man who had skeletal lalom which appeared a area of refaction go raat genogrums. Destruction of the skull in this diseno u derarbed by Kren (139) In this case, the face had becomo monstrously deformed by a confluent tumor which was a herd w curtilago and localized in the granter part of the face and tbe left ddo of the lower jaw After 4,750 roontgou relation, his face bo Chino Dearly normal in stza, Tho skin wu slightly atrophic with sono cnlarged velns, and was fantly bluish Tho do fects of the skull Largely filled out md be felt well. Pomel (203) daxcribed a typical nodulo at tho base of the skull and In the rulcus transversus Jacobreo (125) reported a 5 year old Norwegian woman whoso lesions word bealized on the face. Roentgenograms sbowed Large defects in the frontal and mal bone Bono Involvement has also been described by allerzeelt (169 caso 7) Bomhardt (18) and Pick (1986) Joanaline, et al (198) found bony calification in the patient. Beek (18) descdbod tad cases of Lapod, harcome with well developed clo pbantiasks and lymphectuslao and lymphocytie infiltration of the lymph follicles. The first patient had a large olcerattoo which becerdtated amputation of the leg and tho second do velopod localization of the bome 7 Ilmart Lacolconieat There have been moveral publications in recent years which suggest that Keposts porcoma may cvist as a primary tumor of the boart without cutaneous mandfestations. According to Stats (246) the tumon ore detected dont by their stze, bemorrhago or lortation of a serOS IDEMbrine loading to an effusion tato serot mo (plowa or pericardium) as to three of his cases the fuld i frequently hemorrhagic and may be anthochromic. There be a trodency torrad recience Axdtes of significant degne have not beca described in one ause in Stats terhes, there were several Dojokes to the picnlum and myocardion of both vcptricles and in another patient, who had atensht ksions, the heut 120 Kapod's Sarcoma vus spared but several tumor plaques were present at the boso of the parletal pericardium. Autopsy in the latter caso re vealed two firm bluish Doduler, 3 mm. in dlometer observed through the eplaardlam Door the posterior descending branch of the left coronary artery and a dinilar podulo scar tho right coronary artery oo tho anterior surface of the heart Although Weller (275) believe there have been so account of primary beart Involvement in Lapool's percoma "as ruch, It Is his opinion that it can occur when the histological descrip tion of e cardiac Dooplasm is characterized by sarcomatous tissuo infiltrated with severe hemorrhage, It is suggestivo of this type of involvedcat. Ho reports two cases which were diagnosed at autopry The first patient, a 30 year old man, Arst developed cough, yellowish sputum and night sweets, together with adema of tho face and mild faundice. Romnt genogram of the chest revealed a vertical shadow in the night auncular region. Autopsy revealed ocarly completo occlusion of the right aurice by a large mass which was firmly attached to the wall. The initial symptoms in the second cuc, a 90 year old man, were weakness and cough. At autopay the right auricke revcaled a bemortbagle peoplastic mous. The char acteristic physcal dgn lo both cases, According to Weller was the peculiar evena of the face and deck which, despite tho apparently normal testure, appeared war like om stretched over an increased amount of subcutancous tissuo In the patients described by Chodset ADJ Ramsey (10) the process originated in the skin and one patient Hed as a result of betrotthage from a visceral lesion. They believe the reoplasm to be a CADOLTOU representative of vascular tumors and should be designated anglosarcome while tho cutaneous subvariety should be called Lapost's sarcoma. The found sections from the Cabot case (38) lo be kipatial with thickr own and therefore believe that polmarv kaposis sarcoma of the heart spot rare and attribute prestou diagnostic al Sculty to lack of proper identification Shinilar cases have been described by Aegerter and Palo (2) and Cuhne Vlotta (50) although none of these patients Symptoms 191 ohibited a distribution of laulons Amilar to that of classical Kaport's mrcome with visceral involvement In thoso cases, diagnods is determinod by the hbstological resemblance of tho ardiac tumors with the common cutaneous tuman Ac cording to Hamilton-Paterson and Castleden (107) 2 rocalled preudonytom of the beart may havo a similar structure Stats (210) belioves that cardiac involvtuntent in Kepod's sarcomatods with cutanoow fovolvement diffen grotly fraan these C Hortata that people to lovalveient of the beart in Kopod's sarcoma i focidental and does not give nao to sympturas. A 43 year old woman, descrlbed by Raw (206) had chest pain, dyyppa an action and orthopnea for three and odohalf years. Examination revealed arctten, edorna of the legs, enlargement of the superfdal thoracic velns, dullneo of the right chest and hepatomegaly At autopsy the inferior vena cavu, at the catrance to the right anrick, was found to bo toro and a hard, 3 by 3 loch, mercomatoos turnor tovolved tho Hght quricle and eatended down the course of the fnferior VERA an, terminating on the under surface of the liver ua distinct, hard nodular man. Nortoos (181) patient, a 29 yper old man, developed cough and bloody sputum Ho had moderato cyanosis and do cardiac absconalities on phydal Gamination. Autopsy revealed a tunor which fillod tho entire heft auricle atrading down into the mitral orlice which was, histologically Kaposi's sarcoma. Cardiac Involvemcat in a 33 year old woman who had at tacks of substernal oppression and breathlesses for 17 months and cough fever and chest pains for seven months us reported (Cubot Case 22-101 8) Tbo bbulated mus in the right worlcular region, domonstrated roentgenologicaly prored to be kapods urcoma on histological examination Cons of this type hasc ako bera descobed by Clerc, et al (19) Blodet (23) and Elenberg (60) Cardiac malicient with formation of an auricular thrumbu was described by Nesbitt et al (176) Tumor nodula in the seardhm Mere reported by Symmers (la) and 1 Kaport's Sorcoma small nodulo was present in the subepicardium of the left ventricle in a patent reported by Dilard and Weldman (53) Tho lesions were Ilmited to the pericardium in Van Cleve and Hellwigs (265) case. Cardiac neoplasms irere also described by Baculoglu, ct al (9) Crillo (10) Heuer and icmp (117) Vuller (175) Shelburno (235) Smith (212) and wmiw (281) 8. Raro lacalcomani. Vuscles Vascular lesions appear to be raro and the Amooth and strated musculaturo appear to be resistant to the procuu. Denset and Leopold (51) have described involviment of the diaphragin and intercostal muscles and involvement of the proas musclo vas reported by Greppi and Betton (97) There are no reports of utcribe tovolvement Howaret Stads (216) desembed two patients with tovolvement of the broad ligament near the uterus who had tino firm tumor polulos, 3 mons. In dimeter and several dmular noulules in the dlphragm Centrul Icrow Sijaton Bruin lesions have been reported bx Nesbutt, et al (178) and Schtsted and Burkhardt (224) Involvement of the dure was supposedly present in Denze and Leopolds (51) cose There has been no report of spinal cord Involvement Wule fairly typical, Saphics (219) patient Hloued estension along the nerve trunk and blood vessel and involvement of the notio sheath, winch was Hddkd with DIOTOW blood spaces, and cutensivo infiltration of the con pective tissue with spindle and plasma cells. The only other report of this form of Mtensioo in Loports sarcoma wus bv SATXOW (931) Eye Incollement Ocular foyd count is rare in huposis sarcoma An B1 year all man prekated bn Sihat (239) de- Icloped and hemontagie cyst of the night loute conjunctival me which we listologicall reported to he anglosarcoma In addition to the cLusical cutaneous change of deposis Arcos, Sotos (112) patient huu Insals cment of both on Symptoms 103 Thero were vascular ledoms of laconer type on the bulbur conjunctiva and marted pigmentary deposts, with a soft out Hns, ao the surface of the Iris, crossed by several goall, dilated verrels. Adrenal Incalcement A distract, diffuse type of bypaplarla of lipold-poor cortes ou described by Becker and Thatchar (156) Thero were many dark brown eroddons which tended to folow tho mocowl fokls in tho gastrointestinal tract. How CT no tumors were detocted histologically Adrenal corte Involvement was also described by Stets (248) E Agociated with Other Dicota Lytophoma Cootemporary investigators tend to include Lapouls rooma in the lymphoblastoma group of documes. Tho trotuing number of reports of Kaposi urcome a sochatod with members of this group se evidence in apport of the relationship of this discuso to the reticuloendothelial system. Willis (280) ruggestod that Kapool's furcome may bo a variant of the 'lympboblastomes with prominent cu taneous lesions related to those of Hodgklo, dlacuso and mycool fungoddes. Hycosts Fungoida. Histologically confirmed Kapods war come which occurred late to the course of mycool fungoidon Wu reported by Lope and Groerwoud (113) The mycosts fungoides, of four years dundan, had evolved through the profungokal stage to the typical tumor stage. Numerous small, round dome shaped noapłgoxented tumor appoarod on tho feet and gradually mtended up to leg. The previous his tologic diagnosis was mycosis fungoides but these trutors showed histological changes typical of posts arcoing, with conserably les pigmentation than wol Post mortem rec tots rocaled Kapood's narcoma and typol mycosis fun goldes In the fine section. Laponth (148) reported a case in which the lesions on the tocs Here Kaposfs sartonna while those on the trunk weye micosis fungoddes VVinet (283.core +) re- 124 kapod's Sarcoma ported a 76 year old woman who had myrosh fungoides and Kepod's sarcoma. Hlodgkins Dlacas A 65 year old man who had hepatosplo- Domegaly lyruplladenopathy remittent fover weight loss and slight leukocy tods for four years, hus described by Goldschlag (90) The diagnosis was malignant lympbogranulomatose. Pigmcated raised sodules appeared on tho Lowet optremities and right side of the thorax thrco and ono-half years after tho anset of symptoms. The gros eod microscopic characteristics of these nodules indicated post's sarcoma, Goldschlag pro stined that the occurrence of tiro lymphatic system discasco in the same patient was the result of the samo stimolus. Tal bott (256) described a 50 year old women with Hodgkins discuso lo whom superidol lymphocksopathy was perdal by entdence of Lapod's carcoma of the shto Tho chronologkcal association of the two discases wus fality claso but tho culo logic association is difficult to cablish A 51 year old man who developed Hodgkins discuso nino months before the appearance of cutaneous lesions of Leposts Larcoma, to reported by Greensten and Constop (98) Tho pathologic dlagnoses at autops WeTC 1) Hodgkin carcoma of tho spleen, hmph nodes and bone marrony with puloronary and renal iniltrations, and 2) Cytancov anglo-endothellorna (Kapod's sarcoma with lymph node and pulononary mctas tases) It is their belief that the two conditions present in the patient are histogmetically related and that possible other coincident occurrence of these two lesions haut pot been fully appreciated and recorded. Osborne et al (187 care 7) reported the association of Kaposi's sarcome And Hodgkins discuse in a 67 year ok? woman. This association has also bert absetia b VicCarthy and Pack (153) Erf (68) and Ilolf (259) Lymphosarcomo A paticot hesiog Kaposi's sarcoma luch cescloped into hmoplosarcome was reported by Bellow (18) VícCarthy and Puch (153) described tud cost of hapost's sarcours one of which was assocated with Bnil SummeTS Symptome 195 disease and the other with lytophotroom Higglos (118) ro ported Kapodi Coma of the skin and lymphomarcant of the lymph nodes Ho belove this does not represent a rep Bruto disso entity but rather another of the verlod man! festettony af Kepod't wrcome Bluefarb end Webster (280) reported Kaposi's tarCOMA podated with lymphosarcoma (see Fig. 2) This 78 year old Negro had been bospitalisad on several occadons bocuse of lympbosarcome of the lymph nodes. Following roentgen ny therapy to the cervical, sternal and abdominal arous, tho lymph Dodes gradually docteurd in size, and bu gepera hoalth inproved. Seven months later he developed swelling of, and a for nodules at the base of the penis. Several months later a mall bump appeared on the night thigh. There wero Dumcrow reddib-brown to bhio modulo varying from 1 mm. to 2 cm, in diameter Sonne woro alightly induratod and this induration catended to the right tognina Mita and fipod with the Ughtly enlargod loguinal lymph nodes Tho aldo was bolewhat hyperpigmented to the involved tree. There wa duffute swelling of the penis with a few small indaryted popular at the base and hyperpigmentation of the overlyfog skla Turo hyperpigmented patches, with central couting were prescat aboro the left leo, optelorly moderately colasged lymph node was present fin the right awlo. Lymphatic Leukemia Cole and Crump (H) described a 63 year old man who had dumeroa, lorrgularly shaped arous of brownish red and bhaish red pigmentation scattered over the entire body and most merhed on the stremities. This condition progressed for three yet til tho papilomatow over growth on the lower lp and left foot medo walking la possible. At thl time, dood studies realed hytophatic Luhtala although the cutaneow lesions WeTo dever chanc beristic of leukemia but were always tatincths of Kapods aircom. The patient recorded by Hufnagel and Dupont (193) was 75 year old man wbo hud microw lyzppbold nodules asociated with the vascular lesions of reports Kapod's Sarcoma marcoma Histologic ovideo.co of Leposts sarcoma and lym phatic leukemia in the same lesion is described by Sachs and Cny (217b) An eruption, consisting of Integularly shaped violaceous plaques, 1 to 4 cms. In diameter had been present on the left sole, Instep and inner espect of the becl for seven months. Histologic otamination revealed Kapod's sarcoma in tho middle and upper layers of tho cutis and lymphatic leu hemia in the deep cutts. The clinical features of Kaposi's ur coma and the hematological picture of loulcmla w co present In all three of these petlents and all three died of leukemia rather than of heposis sarcoma. 01 heposti aroma associated with h mphabe Leckerli. QZ hipoals wartood associated with hmphatic Leukeola The simultaneow occurrcoce of Kaposi's worcone, lymphatic leukania and diabetes is described by Fischer and Cohen (74) The patient, a 74 year old man, hed Laposi's sarcoma for 25 years while the leulenia developed later We have obsctie a patient having Laposis wrcoms and lymphatic kulcnla ulo had involvemeat of the fingers and toes. This man died of himphatic leukemua rather than a Kaposi's sarcoma Vyclo Leukemia The association of myelotl kukemia and Laposi's sarcoma ku reported by Tedeschi, ct al (257) Symptom 127 This patiat, 265 year old woman, had oumerou purpudo 2B scattered over the trunk and ans. The Kaposi's ar COROS consisted of a modlardinal mass compored mainly of endothelial-Iined Anason and of impervions or analized endothellal sprout. There structures were embedded in a fibro cellular strome which displayed widespread prolforation of reticuluma cells ad fiber, fibroblasts and immature bepatic cell. There were concomitant red blood cell otravusations, Add blood pigmoet wu moted in places Colleret und Gallet (51) reported a case of Kaposi's a Code with myelold lakenla ta = 74 year old Italian man The peripheral blood count abowed $5 per cent polymorpho nuclear leukocytes, 22 per cent motornyelocytes and 6 por cent myelocyte. The sternal bone marrow emination rovealed 57.5 per cent Deutrophila candsting of 13.5 per cent polymorphonoclears, 20 per cent metamyalocytes 18.5 per cent my clocytes and 8 per cent procyelocytes. Caranoma A 67 year old woman, described by Stats (218) had nodules of Kupoat's mircune affecting both lower extremitles. At antopy a finca, ulcerated, fungating adenocarcinoma was found in the sigmoid colon Ebert and Otruka (9) prosented 0 year old man who bad Kaposl's marcada involving the toes and dark of the feet in addition to carcinom of the rectosigmold region of the colon The 73 year old man, roported by Binder (24) had an adenocarcinoma of the stomich, which was removed surglaully md approximately si months later developed Kapost's wrcome of the right ankle. Diland and Weldman (53) desenbal Kaposi's sarcoma asoch ated with a hypenephrome Indreis (GA) and Carpino and Secchi (35) bave reported Keposi's wartoe nirociated with fibrosarcome. Abramowitz (1) described . 00 year old man who had Kaposi's sarcoma and osteogenic sarcoms of the tilbu. Philpoons (197) patient hed Kaposi's sarcana assot ated with widespread ourdoma which was posmary in the Hier The patient reported by Silver (239) had Kepoat's wrooms, Habetes and cpithelboma of the fact llo observed a 54 year old Italian woman who ha Kapools 108 Kapod's Sarcoing Larcoma and adalocarcinoma Tho ledons on the right leg which had been present for three years, werd pigmented and thero ww one crusted, purplish podalo the size of a chaty Tbo uterus had been removed suglcally four years proviously because of adenocarcinone of tho curv, 63. Lapod's sarcoma in a patient with carcinom of the ccTvIL. Other Discant. Lorosis murcome associated with yoo Rock logluuseos disease of the skeletal and now system was described by Rogrt and Vigne (203) A patient having Laposi's sarcoma of the legs and lupus vulgaris involving the boso was reported by Eller and hest (63) A case of hapos i corona associated with hklraclenius supporativa was presented by Blumentual, c al (27) Diabetes Jfcllitus. Ad incidence of 18 per cent diabetes meites, of alt of 13 patients having Lapossa sanoma, noted by Huolbut and Lincolo (121) Two of these patients WTTO lacun to be diabetic prior to the diagnosis of Laposts Symptoms 189 Orla and the otha four shoved evideo.co of diabetos following the appearance of the cutaneous lescous. They believe that patients with Keport's mrcome should be studiod for possiblo dlabetes. The reason for the coordstance of the two dheues ww Dot apparent, and they found tho adminletration of arxentc did not appear to affoct the cutaneous lesions Tho posibility of an endocino imbalance is suggested to co Instanco (124.a. 2) and although a diagnosis of diabetos wu not made in this case, the results of the glucose tolermco text and the patients adlization of high doses of losulin rug gested the presenco af en abnormal and insulin factor Definito dhnical tmprovement followed the omset of spontaneou fem inizing clunges. Since this disease Occun moch less frequent ly in women, they bellovo further study frorn en ondocrin ologlo standpoint, should bo donc, Fischer and Cohes (74) patient, a 72 year old man with Kapod's urcoma of tho stremities of 24 year duration, subrequently doveloped lymphatic leukemia and diabetes mellitin. The patient described by Silver (938) hed Kaposi's wrcoma and diabetes.
VI DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS T.. UC CLINICAL dlagnostic features of Kapoor's sarcoma, us out kned by Roncheso and Kern (210b) are 1) Tho liseuse octur most commonly hnen, 3) The greatest incldcaco is In the slith and soventh decados of life, 3) The atromide arc predominantly tovolved, 4) Tho distribution is usually symmetrical, 5) The lesions condst of blush, blualı ned or reddish brown solules anxl plaques, 8) Tho Lestona arc piln less and ulceradon is uncommon, 7) Edema of an extremity is common, 8) Tho residual atrophy and pigmentation of the lesions involuto spontancovaly 9) The course of the discuso is slow and 10) There is no change in the patients general condition unul Lato in tho diecas The Dumerous diseases to be considered in the differential diagnosis depend upon the charactcrlxlc fcaturo, or fcatures, which are present Although the clinical picture is sctv tv7)- Ical lo this condition, it has occasionally been mistaken for Bocchs sarcoid, crocketma pigmentosum, acanthosis nigricans lichco plapus, granuloma pyogenicum, glomus tuoor syphilly Leprosy angiomas, anglokeratos's my costs fungoides und nxeta Hatc lctiam SunCTS (25-la) believed tio iclangiectauc purpurtc lm orrhagic and simple inflammatory processes should be iller entlated to the early phase of Leposi's sarcoma, before the Occuntcr of metale hyperplish Tulangetusta, he con Uniks, is not related to in perplade of blood and lymph seseb lett vessels, particularly apillaries are present in Input plasia, while there are dilated, tortuous seseb in telingler tasia. Hemorrhage is not a diferential point in the cash coure 130
Diagnosis and Differential Diagnosis
81 Losion resembling angomas and granuloma pyogenicum
(Courtesy of Franceso Roncho AD)
85 Lasom roembling anghomas and granuloma pyogenicum
(Courtesy of Francesco Ronctions, BID)
131
13 kaposi's sarcoma of Kaposi's sarcoma unless combined with other features of the disease. A concentration of cytology" plasma colls, angloblasts and spindle cells is important sloco these cells together with vascular hyperplasia and bemorhagado not occur lo a simplo infiammatory procesi According to Vaches and Cipollaro (1555) tho clinical diagnosis can usually be quickly and easily established, acept in various stages of avolution when the disease may simulato 66 Luons resembling ungomas and granuloma Mogerixum. (Courtesy of Francesco Rooctiae, AD) lichen plenus, mycosis fungoldes, svphille morooddosis, or lep rosy Lichen pluous, particularly the hypertrophic type, closely simulates the lesions of kaposi's sarcoma Leslau occuring in the prefungold stage of mycosis fungoida resemble variou scal and cultlic dermatoses but the issions have no pro dikction for the stretnities. Sypuls is diferentiated by the history distribution of lesions, raw ham color response to therapy and scrology Although marco dosts may simulate Tupost's wrcoma for a time, thus condition presents no Ini trated plaques, vascular elements visceral lesions or definite proclicction The brown colored lesions present in legyrosy Dlognor's and Differential Dlagnons 133 have a slovrer evolution and a loog incubation pardod. The unathedde present in leprosy is never encountered in Keports Promo With definite hyperplasia of the regular conectivo tamo or other cellular elements, some of tho granulomu ud nooplass must be madudod, according to Sacha, a oL (2172) Tuberculosis may be excluded by the absence of epithebold and grant cells and tubercles. Syphilis can be eltiningtod by the presence of angloblouts and spindlo cell and the Abeance of vascular changes and plasma cell collarets. Thero is a difference to the datelopment and evolution of greonlattoo Haruo which terminates in comploto fibroals and there are cells which aro not present in Kapods Arcoma Thero i utally an abundusco of flbroblasts and place calle, whilo angloblasts are scarce and there are no spodle cell. They believo the most frequcot and important peopleans to be considered uo angloon, Roglosarcoms, spindle cell ercom, globus tumor granuloma pyogenkum and drcumscribed lymphangoma Tho condon, typical anglomu caur littlo diagnostic dibculty unless thero o cellular elemento soch a angloblaste Thoy believe this type of englana is frequently referred to endothelioma or peritheliome, although they are in agreement with those who question the tom peritbekovna and believe them to be englomes associated with angloblasts. The differential diagoods of these anglomas L established by de absence of honorrhago and godlo cela. A positive Perls reaction plasma calls and troused hymphatic vetsels and spaces does not occur in anglosarcoma. Lacking these feahren, Kapool's sarcoma i differentiated from anglosarcome only bry proper diagnosis of the initial leston. There is little diffon between spipulle cell urcoma developing from Kapood's sar coma or from an umrelated condition. The proper dlagros MLIV OCTsoonally be established by the presence of angloblasts and plasma cella Corroborating aidence of Lapod's sarcome, wuch us the dinical picture wd history may establish tho dlagoon's Histologcally glomu tudors show the capillaries, enco to 134 Kapouts Sarcome the form of anglomata, to held a surroundlog Intenso, uni form focal infltration of angioblasts. Other types of cells uro not found Tho partidpation of nervo ond muscle tisto to this process is not stressed according to Sachs et al (217) They state that typical ances of glomus tumor are found histopathologically in clinical and microscopically proved cases of hapoods surcom. Proper degnosis can bo established only by careful study of the entiro section. A positivo Perls reaction gecay occur in Leposl's sorcom. Although it should be presumed that bemoddala would also bo present in glonius tumon and granuloma pyogenicum they were able to demonstrato its presence in only a small percentage of cases. Tho patient descobed by Klabc (135a) appeared to have Fomo chincal fcatures of acroder atitis chronica atrophicans or of an tolerma crythematodes of Jadabohn although the absence of clinito clastic atrophy did not favor a dlagixosds of either of these rolated conditions. This patient, a 50 year old man, hal developed erythema and oderna of the back of the harkls de veurs previously There were secral circut Kabeh soul edematow areas lovolving the corsun of both feet and lower legs. There was an intense ctythematous edama of the luchs of the hands and flogen and similar although muller discrete lesions on the Betor aspect of tho wists and forcenns. Cuchrist (8 a) obscrved a man havtng a typical rosicca of the nose which proved to be Lapost's garconna on histolog ical cramination Lesions appeared on the atomic a fer years later und the case became one of classical Kaposis ALTCOULA 12vcar old man, karlbed by Nics (170) hul deeloped a flat purplish red arca on the lower log > months previously This losion gradually colarged became hand, clevated ad Harti Amulating hypx trophic lichen planus Bancrofts (11) patient was a 49 year old man whose initial lesions mund on the left ankle anal later involved both legs. The lakens appeared licht and simulated Uchen plonus. A smolar capo dmulating hyperemphic lichen planus was presented by Dlagnor and Differential Diagnosis 135 Combleet (10) Zeisler (290) stated that this resemblance to sometima striking and he had obtained two patients haviog typical Laposi's sarcoma who were being tronted for Hchen planus Although granulona pyogenicum and Kapod's arcome pro kent similar vascular changes, tho cytology diffar. There are numcro, diffuseły utangad angloblasts placna cells are not promtrent except in secondarily tnfected laddams, and there are no spindle cella Thao Httle tendency torvard Abrons in granulomn pyogoniam Wigloy (278) described a 13 year old woman who doveloped a painful, red, semi-solid "angiom atou tudor on the ball of the foot. This lesion, of Albert nut ate, wu flush with the skin surfaco and simulated granu kuna pyogenicum. The 40 year old man, reported by Becker and Obermoyas (182) hed recwtent erythomatous, elevated tumor on the chin for four or five year Despite cauterization, the lesiona reconed Somo of the lesions vero peduncu lated and appeared to be typical of granuloma progenicul.
VII PROGNOSIS SONE Joure lavestigators, lacluding Kepod (132a) bellove this disease to be fatal to tad to three years while others are of the opiniou that the duration may be egbt to ten years, and rare casca may survive for 90 or more yeurt. Severs, rapidly progressivo casos, having a sudden fatal outcounc bave been described, particularly by Italian ob- SCTVCTS. From the reports it woull appear that this discuso has a Doro violent courso among Southern Europeona. llant son (109) believes tho tumors are more malignant in younger than in older persons, although bis ovidence was not con dudie The prognosds shouki bo guarda, according to IcCarthy And Pack (153) doce long remiolons are quite deceptive to both patient and physcan. It is difficult to onluuto tho ag gressiveness of the tumor for many months and deceptive it. missions and bizarre variations may occur in WuDCT Tiry Also mention unusual rucial influence the fulminating tvpo betog estremely rare in Scanlinavians and frequent in Italiano and Jews. Generally the prognosis is not favorable when there to raptu tumor imoletdent of both entremities, asboclated with alcma, or when pnman or secondary lymph node or visceral in ohiment occurs Thy formd no relation of the age of the patient to the clinical activity of the disease and death wwwlly resulted from progress ir cacheria intercurrent infection or hemorrhages from friable bull: tumors in the fustrointestinal tract or lungs Septicemia and gangnite following rouince cos, may ako be the chief cause of death Among 38 patients stulkd by McCarthi and Pack (153) 28 Wero living and only sett hete ime of the disease Three 138 Prognode 137 had been well for five, di ond nine years, respectively e defin Ito curo rato of 10 per cent of patients treated more than five your provlously Four of tho group of living patients, however had survived 10, 11, 12 and 25 years with actiro direase. All hed roodved radiation therapy In tho sades of patients who died of the disease, three had survived five to ten years, and two had lived for 15 to 20 years. One of theto patients died of prostatic cancer d years after the titlation of therapy for Kapood, Arcome. The averago survival intarvel, with treatment, was approodmately eight year Tbo verlation to duration appeared to be dependent on tho age of the patient in the causes Audied by Douffel (58) Tho younger and okler age groups had a more rapid and progres dve coun Ho noted many recorded areas of 85 yan dura Hon und found the duration to be from cght month, u do solbed by Vlorlani (160) to 5 years, a reported by Prolopcuk and Cutalon (200) Ho aloo found that the race of the patient had to boaring on the progDoda. Most patients died as a result of prolonged progressive wosting and emada tion, although some died of Intercutent disease The course is ordinarily slow and steadily a intermittently progressiva, accordtog to Vackeo and Cipollaro (155b) who give the duration v ode to 25 years, with a five yeur average. Chaluser and Ramky (40) found the duration to bo si months to two yeus to the 13 fatal chres recorded in a seven year perlod. Death muy ocow from infiltration of the lungi, the or pleen, but Involvement of the intestinal mucosa with ukatation ausing hemorrhage and esanguration is much more frequent, according to Argenter and Peale (8) When the tumor is primary to the vborta, cutanaw tuon wally are not present. The disease ran in tremely rapid course in a 63 yar old Homan described by Simmons (522) Kaposi's sarcina fnvohed the grater portion of the upper entremities and appeared as scattered Dodules another body nueces, including the face and scalp. Deuth, resulting from fotcrcurrent cawa, occurred one month after the appearance of cutaneous pod 138 Laposi's sarcoma ules. Although Symannens had never known upool's sarcotia to occur in a patient with jaundics, ho suggests that hemor chage into the nodules may bevo beco encouraged or fitlated by bilo molts. haposl (1322) described patients who died after two to dry or aght years, who exhibited secondary deposits in the internal orgams, particularly the largo intestines Howter many cases having a similar onset have been reported which were obsened for long periods and did not prove fatal. In 000 such cute, described by Braytop (99) the patient had been well for 25 years and Vachenzes (150) patient ans well 15 y can after onset of the disease, although the local severity of tho discaso had necessitated amputation of one leg. A 70 year old man, reported by Silvers (0291) had the disease for 15 years Estrios (67) patient, a 68 year old man, had edcme of the leg for 35 year but the condition had been painful onh for the first 10 years. Two years before cremination, nodula dom cloped on the left foot and spread slowly to inoho the entire Leg Estrio docs not believe this 35 year duration to be unusual in Kapod's wrcome Sulzberger (253) reported a case of 18 to 20 vrars duration and Zakoos (2691) patient had the disease for 25 years. A 53 rear okl man, described by Highman (120a ) had lesions of Kapod's sarcoma for 3 years Tho 53 year old Greck man reported by Akwa, et al (1) lud Lapod's sarcoma for 13 voan and turn of Hanssons (103) patients, who developed the disease before they HTTC 50 years of age had been symptom free for 10 and 24 yren respec tiels kaposi (132) Belies ed spontancow healing of the tumors pas due to hemorrhage and fibrin deposits with subsequent organization and contraction Hounty Philippson (197) and Dall Fustra (52) believe that henronthages are pot directly related to involution of the tumors and thrin deposits rarch occur Cuchrist and hetron (87b) suggest that the insolu too is due to gruw blocking of the small arteries, cupph ing the Losions with sclerotic processes. They bellone tho more vegetallic the growth, the greater is the amount of Wood Prognose 139 roquired. As the blood supply i decreased, tho tlouo grad tally roet to the more resistent fibrous type with completo involution occurring late in conto cues. In cases having a nettoned source of blood supply us in pedunculated tuman, degenerative changes may occur in the new growth. A case of "Pigmented Neoplasm of the Sun" wa deteribed by Hardaway (109a) The patlont had muldple cutaneous tumor for ten youn which were histologdoally alveolar wr comatu. The general bealth was not affected. A later report (109b) states that the patient vous in good health 15 o 16 years after one of the done and the pumatow thano had wdergono involution resulting to a nearly atrophic condition of tho ilin The patient reported by Viller (170) hed developed many ledors of Kepod's sarcoma of tho foet, handy and bent about 10 yean proviously Three years later a Loreto streptococcic infection of the upper arm developed with fever of 100 to 107 deg. F for a considcrubla period of time. TWO months later the lupoes hercome lessons began to disappear and within five months wero enbarely gone. Sú years later there was no recurrence. Formans (760) patients who had the discuo for 3 years, showed a morked tendency to spoutarxous cum The progrods of Kapod's sarcoma appear to bo unfavor able despite tho fact that spontaneous auru, apedally clo lcal curey following therapy have been reported. Spontaneou healing was doted by Bernhardt (19) Brayton (99) Dalla Foleta (53) Gacurist and Ectrom (875) Hantson (108) Heinam (115) Kaposl (32) Blackce (15) Philippo (197) Sellet (230) Viso (2582) and others. Despite the oumerou reports of disoppaanscc of the lesions, without recurrence, Vackce and Cipollaro (155a) bellero that eventual recurrence the rule. Tbe treated eruption Duy undergo involution but there is little or no influence on the cause of the disease and it does not present the developont of new keylons on other areas of the body Although muliatoo may occasion appear to result in . saningly PeTruxot regression, Kulchar (141) bellesa there are unal hurence VIII TREATMENT Radiation Therapy There is general agreement that roent gez ray therapy is the method of choice in the treatment of Lapoxlu sarcoma dnce tho leslows are relatively radio-sensitive This therapy is particularly effective if the lesions are multiple or if the process is diffusely otenstvo The early more vascular macules and Doduks are far more radiostustive than the older infiltrateci plaques, according to McCarthy and Pack (153) These corly lesiotis will wually regreso completely when treated with unfiltered low voltage roentgen rays (one to two threshoki shin erytheo) For larget superficial arc., unfiltered low voltage cose's (100 Lika volt, 20 to 30 cm target skin distanco and small portals) for an average of 100 to 800 roentgea tulividual dose, u mug gested They found that largem doses, with some fltration, were required for bulky skin plaques. High voltage roentgen raus ucte administered to deep tumors which involved lymph nodes meillastinum, lung, abulominal viscera and bone In two patients having the disease in the os calets and tabla, Ux turtors regnsel and pain disappeared follou ing 200 Lilo olu of rocatgeo rant. However with the morption of one (lra matic regression in the treatment of splenomegah the found high volt: roentge may therapy only modcratchy sucassful. They strongh emplastze that radiation therapy shortll be administered in constratiis closes if the circome fototne or edhema is present, stoce the blood supply is usually impural Oortreatment may cause cos's, Infection and fun TTS Interstitial radiation and blanket rodation with Large porta, are hazardous for the same reuon Tire cutaneous lesions of posis Wrcome are occasionally 110 Treatment recakttrant but wwally respaod favorably to doses of moontgen rays which can be administered with safety recording to dlackee anal polaro (1536) Largo doses should not be admiloistered over large areas wpecially to yong middle agod patients, mm mono conservative meanire have been tied. To production of erythene should be avoided. They Ruggert tree torrent of the cutaneous lalong with 75 5 Onco woekly to all are involved by the press. Four to 12, or Occasionally 16 trentinents may be required and this amount should not, ordinarily produce mythema Superfidal latons, not too thick, oro tutal with $0 to 100 kiloolis filtered radiation le class of sousually roadstont eruption, radiation should be discontinued and arreole given for several months, after which . dngle crytbeira dose (300 e) or a larger dose, may be adminirtered to all kislons Larget douct may be used for anal lesions Muce the area may be occised in the erot of injury and the effect is less than for largo arou where coxlary and particularly Kattered radiation must be con suere. The effect is approximately 35 per cent greater for a for square loch area than for an area of one square inch, according to MacKee and Cipollaro. Therefore it may be safe to give a so-called trythrina dove, or EVE A mixh larger dore, Io per shee noduko bat urule to administer this mount to a large arr.. Some pigmentation may perust for many months folloxring itsolution of the oruption or there may be atrophy and possibly telangiecture. They suggest the we of Bhere radiation, usually 3 mm of al., with no change fra the douage scheme for the treatment of that letion. They bebunt that israeliation is Kapoufs urcotud acts in much the KUTK momxeT as in mycosis fungoides, a condition which attually cake to be benefited by irritation. This fact kas sxx bxts observed in haposi's sarcoma Guarini (101) described 30 cases of Kapost's wrona treated with tornigen aus and he considered the reults to be en good. Ilanso (103) treate patients xels with radiation. Ile emploved roentget raus to large widesyxcau bestans and infiltrations aux madium on Implantation or an Kapool's Sarcoma plication method to single tumors Twelvo patients wero treated with roentgen raya varying betireen 1000 and 7000 r tho angller doses given to a largor flekl Treatment was ad ministered Lolly in doses of 300 to 500 r euch. Five patient were given filtered mediation (1 mm, al, 100 kv, distanco 23 to 50 cm.) In the other cuses, 4 mm. AL or one half mm. Cu. und 1 mm Al were used as Altar, 150 to 180 X 33 to 50 Cins. distance. The emperes varied from 1 to 6 milhamperas. Some more restant cases wird glimt a second similar series of treatinend two or three months later Theio was a dry although ever desquamatiys, opucrinitis in a fou cores AU tho tumors treated by this methodl disappeared with the a ception of two cases, having insulictent follow up observation, although the showed definite signs of reading. Kulchar (141) belle e small doses, of about 75 r should be given at wcelly Intervals, although larger amounts of filtered radiation may be required for more deeply infltrated lesions. For (S0b) stated that he had better results with Altetah, than with unaltered, rachation. According to aren (139) the best therapy i roentgen firadlation gives alono or in combination with arsemotherapy VIcCarthy and Pack (153) found that one to two threslokal thin crythemas of upAltered low voltage roentgeo roy pro duced a smilar response to that obtained with one to two threshold crvthema doses with radium plaques et 1 an distorco. Small lesions will respond to bets and garmi ray's of radiun Just as they do to rocntgen rays according to Vacker and Cipollaro (1935) Beto rays are seldom used except for Very superficial Lesions with neues Less than 01 nm 1L Lightly Altered (1 mm. brass, 1 mm. al) Hamina saus IT satisfactory for most wall lesions with the applicator in (ontact with the surface or at a distance they bellese houett that radium and randon therapy in not getItalı su actory for Korosis sarcoma Luciuse of the numerous lostom and Large areas to be treated Fhe of Hanssons (109) patients were treated by Implanta Trostrient 13 tion or surface opplication of radion. For implantation, bo used peedia with 10 mg of radium clement, with about latha active length anu a 5 am distasmo between the needles. Txo Infiltration wu quivalent to 1 mm of lead and the deso varied between 10 to 40 mg radium oleacut for hyo is on half to four hour. For enfoco application, the Joso wa 1,50 me rum elemoot for Horvo hours, distanke 19 somn, in Atakkn equivalent to 3 mm lead. An tumors treatel di appeared. He believes that if the tutors and bafiltrationcs are multiple and the arce of implement Largo, roentgen ray therapy is preferred, but sloglo nodulas may be treated by Implantation or surface application of radius. His investiga- Hans revealed no factors which might provo aoo melhor more efficaclous than the other However ho makes no reservation ha regard to raciun løplantation in honors of the legs and feet, ce no patient developed Doccios' following a modes Ale dose. Ilare, al (110) egiplayed high energy cathode razt for treatment of cutat US lympboanas and Laport's sarcoma. They believe the clinical advantages of this type of therapy to be the possibility of selecting the derired electron penetru tlon by control of the voltage the compacte absence of damage to tarus bond the wel canal election range and the srund beologic effect in the outer layers of radio-serative Ultracolel Lichi Therapy Jacobsen (193) benet that treuemnt with Firstu light and quarta light have gheat couraging results. Swygkal Therapy. There has been little reference to, o et perxTKT with ske mrgkal trest of Kapodo urcona. SloCartly and Pack (152) head gratthing results with whale Serekalencision of marh olitar lesions. Amag wat sch Ikent o use free of the discuse frie hai cecision of the lesson at the onset of the disease. One patient was well efter nine muss mod two patients whose priman cucule of the panks had been treated by amputation, were well for bie and
Kaposi's Sarcoma
6. Cuturous lison of the foot before cotbocker therapy
(Cowtery of Francesco Ronchose VI.D)
6. Cutaneou kinons of the foot follow ng callcode ray therapy
(Courtesy of Francesco Roachese VID)
Treatment 145 six years, respectively Surgery is contraindicated in cur having attend vo multiplo tumors or if there is an associated adama Suth deana wally indicatos deep veskel involvo meat and prodispores the true to Infoction and indolent postoperativo dofocta Funguting tumors of the rul cavity may occasionally bo rezuloved by pallatiro autery exctaion and should be followed by botrural roentgen ny therapy Small lesions may bo removed with the scalpel or with the cutting current, or they may be destroyed with electrodesic autkan, electrocoagulation or other forms of electropurgery according to Blackco and Cipollaro (1556) Nalther acidom por electrocoagulation prevent rocuted.co or spread, accord ing to Jacobsen (125) although scoglcal intervention may bo indicated in ouren of puppuration, gungroo or ortenstve do struction of the bodo Chomical Therapy Nitrogen mustard hus proved disappointing in tho few patients treated this for us mentioood by Onborno, et al (187) Seagrave (28) and Wintrobo and Huguloy (281) Michelson (168) reported fallure with urethano therapy for Kaposi's sarcoma Although penkeillio has been reported of valuo in the treat ment of Lapod, morcom by Grinspan (100) and others, wo have had no success with the administration of penicilin, or any other wtblatis, in the treatment of this disease. Bloom (95b) reported that two patients treated with otro ucular cytotode serum of Bogomolets failed to rapood to therapy Treatinct with Chloroqain and steroids has recently been advocated but the results do not appear to be favoruble. Astonical Therapy Arsenic in the form of solution of potat stum arsenito a tho intramuscular to ſection of solution of podium ansetato in occasionally efectho and should bo wed B an aclfoveot to frradiation, according to Kulcher (141) J.ucobs (195) abo believes ensends, in the form of neon pheunioe, often appoars to be noticeably effective in audog the cutaneous lesions to disappear and to preventing local ficuTCROC 1-16 kaposts Sarcoma Although many believe arsenic therapy to bo of value, como consider it to be of questionablo value. This treatment was used more frequently in tho past then toulay and Vackco and Cipollaro (155b) found do statistics, based on a reasonably large number of cases, with an adequate observation polod, which showed impressivo result. Horrover many patients in tho puzst wero mdoubtodly benefited by this treatment. This drug should be used, they believe, together with roentgen ray therapy or in the event the latter fails. Arsenio is usually administered orally elther us Fowless solution of Aslatic palls, sod alto by subcutaneous, intramuscular and otra venous infection Results are approa imately the come with the various methods of administration. Best results aro obtained by intendi o treatment, they believe, and they prefer tho subcutaneous fojection of per cent sodlum arsenats, daily If possible, beginning with one drop and increasing one drop at each injection until a dose of 20 or more drops to reachel A rest Interval of at least one month is then given. This treat ment is given in courses in accordance with indications and the patient is cercfully oliseried for estdence of intolerant Contraindications and warning signs are albumlouria, faumdice darbes and other gastrointestinal symptoms, eacher marked Aneni, coogestion of the cyes and any oe cutancow eruption not attributablo to Kaposi's sarcoma In summary nearly all molalities of therapy including et dsloo with scalpel and cautery madiwn pack, cyternal and Internal radon, cootect roentgen rays and 100 200 and 210 Lilovolt roentgen rays ultraviolet rays, arsetuc, antibiotkos and steroids, have been tried without maricu su 11, in the treatment of Laposi's sarcoma.
REFERENCES 1 ADAMOWI, ET Kupoes ICOM with stroruong kroom of the tubim. A. Arch Dernet & S 73 (Feb.) 1922 LATOT, EE AND PEAL AL PAS wocom, . attal ALTE Arch. Path, 34413 (46) 10-12 3.AOOITA, G Eboko md pathopes of scaled cutaneous contode Cim. Chi 39-511, 1838. Abd. Am J Cancer 32. 10, 193 AAWA, CAL KANTA JA Hantos, LS Multiple dopattoo hemorlade muce report. Urol. Chern. Rro. 34.167 ( Alar ) 1950 ANDORONT HB. AND Dwox, T.B Effect of curation md sem botes on the traduction of tuman bor wh outdoo Eroticos e Cancer laut, 45 (Juno) 1017 G. ADECC. A cute for ducou (KAPORI RICOLOST) AMA Dermat o Sypi 2-349 (Sept.) 1632 b ANIMO, C.C. Makiple xbopathie bomoctuple wercome of I pok. Ibu 3152 (Sept.) 1938 C Acum, CC Dion of the She Ed. Philadelphia Sau dor 1954 P 02 7 ALAUZ S FELLO, ) AND LOTTO, D Un cto de mercato de Repoucou metotas prococok bucolarpu Spon made 1 1784 (7) 1934 . BABA 1 ZonMans Medboat dhe praction rohalolu u Throue Lapoy. FCW logel, 168, L 14. part 2 0 173 0 BACALOOLO CLinco CD RATLANU G La theombus Dyodu cha cocer Presse med 41:274 (Dec 20) 103-3. 10 Bumn F ALL PAXD DUTALL Surcorpsbore preth. taltuple de la pero Rectanto de denotti. Bal Soc Inent drndt at wph 10 L 1907 11 B ont L Blwhiple chopathie borba ple mrco (Report) Vitek Deut Sp, A5 (Apr ) 101 BAILE B Kupari opathe beshape arc A. Ilmoch. Br De 09 210 () 185% 13 Bann, BS. AND DAN AL JL spoms der of the petun, report of two A T A L Conto-Urie Sopron, S 102, 1031 118 hapodi's Sarcoma IL Borut PE wpoos wercome with warul features All Arck. Derwoto Supt. 12.321 (Aug ) 1028 15. BOCI, SII AAD OTOWAR, VEI du for du poste (pranu lopa pyoppalcon applouatow Loom beniga Infotou in poma? Lepo 1 urcoma?) Arct. Dermel. Sypiam 31 770 (Alny) 1935 b. BTO, SW AND THATCHD, H.W Vulthplo klimpathito bemar Haglo wrcum o apod, historical resow Dow.cocatore and theodos relative to the Dature of the duck with a ponmootol todo o two cases IRLA Derns, 1 370 (OL) 18:38 18. BOL, CIL About ulo in porotkalamulala hapoy Derma tolapa, 107 131, 1953. 17 BO IL AND Tard, L. Kapod a puluple koputhte humor Hugl mico Act. Det & Smt. +45 (Nor) 101L 18. Balon Li Balochie de Kapod a colutlou lymphawarcowattho. Ann Dorma, a Suph, 9.45, 1010 10. BA ILOT R Surronomia Idiopathic mukollen percotou to (Kupon) Arch / Dennes... Syph, 19 307 1609 Meleto Wwwbluesos ubes were kepenthousin sakinlex PIKDY arom auta ibid 07 237 od 61.239, 1902 20. BOULOK, ET Kpo's Marcoma comud paal wuh pipoco Devu, tot. Dernd Sypr 00 443 (Sept.) 1049 212. BONTACTONI, G Sopa vo ar di mercome bhopatico di Kapod (appo-endoche llama cutaneo) Glor will en della pide 01 590 1820 b BOSTA AL C Stoduo wotopico opna una forma particolare o poly comine di renumolumento di codul mul Mun Lalo patico di Kapad (ango codocheliomu cutaneo) wbud 85 1132. 1024 ¢ BLATATNIC Aoxon nulla urmato clara L Lepout (maloobalothelone cutaneo di Fedeel) Ricerbo a quattro M0v7 D Glor Hol di denn et 0031 103.9. BLAVAC Sartonu de pou to tam ura. ropre Rain wurdt dus e lerep 13 1135, 1912 ANDOR, A E primao vrlon des herros. Ioad Jewich path Cru Ruck 16 417 1011. 24 BIO, AI Idiopath multiple hoorhon wou of Kupe AV Arch Dinud 71 (Out) 1953 254. BLOOD D L by nog op die (0) - BLOOU, D La dixo o Costello (18) 28. BLETTA, SV Voltiple Lopathic hemontage undou Car piron at the Chang Damatoka! Sockty think (Olt) 1955 b. BLAU, SVI \ Floor, IB Oral anualme of the a 119 Reference blood dana. Er, tar tom & Throat Houthly, Log, 182 c Bluorg SV. Allem. Spoes wras swocutod with hophomor. Allt Arch lat Wed, 01-97 (Jo) 19% PLANTASPwd Hat Kapoos betaplape prooma od betredetis app Anak Ders Suph, 6245 Dec) 1850 Bex, CAXD Song, E: Surtoota Hope bicia nubipler hemorthupkna (spod) Anda De L Syphen, 11.00 18 A Burto, ATY Preklad no way of la discotes (kr poudate) 19th mal bathg at the leadepple Why Hedaal Anacletters, Abubnect of the papers and custoulos I was 1215 (lor ) 1803. 30. Bernd, HA Muktuple dopathie heutoeskede urcamos o Expan. And. Doo Sypr, 00..55 (Nor) 1040. 3L Bea LL. Caro o Double Stopalo beunrupo woma. Proc. Nagy Lac Nad (Sat Donat.), 1410 1040-21 32 BCILLATO, C Tocorl deben ped murbo Kapool MARE 22.0 (tor) 1DIA 19 BOCCHAROT W Mates Tapod Dermatologos, 82121 1940 34. Caurte & Abruaroo isce del porto de Repod. Att XXI Cous. Soc. Lud. Orty e Traumatol, (Oct.) 1931 3. Carroco, L. AND SOCI, P. Saraputo cutane boulthplu emor mpar di Kepad Aron ved, 11-361, 1935 Cay Export. Prowery stoot (probly thouTCOR) of the nghat work, CA 91 of the law how to Centul lor pobl Na Lagi Val, 1151042 (Dec. 3) 1930 57 Coro Reports Serpicost of Kepad Leders & Tea of apr preante Dewutloped Conference, bubulol Dry and Cronal Satr Doutologo Arodutos Anck Down Sypt +1894 (Ort.) 1941 11. CATALOG L Dad Mocowe Kepoul cos barrio clasts doute dun repon, soc d. del 1 pH (10.) 1933 Le CHARC, La Lhopathie poltiple botontano wrcoma od koral And Derby, & (Jaby) 1923. b Cauca L La decreasen (2372) # Con, IL A RAVAT ZAL Auctoritkulood culo (por det) o tibor bert Am I ride, S.L. (Her ) 1000 8 ChanL A lo duda od ubed (12) 150 hapods Sarcoma 12 CLON, A. AXD Ohio Un au do turncore myselde ik pont dans Parriletto droite. Ich du do coor 30:381 (June) 1837 43. Cara JC AND ORGAN J.K: Multiple Idiopathio bomoabeple uncome of Kapod. AIA And Derud, 71 018 (31) 1955 4 COU, HY AND CUT ES, Report of txo e of tdbpathie berporrhagic Lancome (Lapool) the first computated sub hmphatic kulanka Arck. Die o Suph 1.483 (la) 1010 45. COX, V Sarcoma di Kupou con durlono attulbaro rcput torto con roperto broxon opku, Glow dd. Dermot « 75 200 1931 18. COM DIT T Rapoas wrcome Arch Dermal. o Syph 39.87* (ov) 1939 Comox, EF AND KAULI FC Kepads nr. Arch Domu o Sypt +0.293 (A) 1911 18 COSTD.LA VI J Kapos sarcoma w kolonythu Arct. Denne! 6 Syph +1 018 (for) 1840 b Contalo Utapon urcure Id 60.073 (Nov ) 10.19 CHOWALL NR Ched by Gilhubst md ketroo (87b) CL A VOTTA, L ILunengo retikulo-schoulori a Fe med Lah Sao rada ] 027 1941 $1 CUAL.LT P AND GALLIT J An pour om de hupou a cha Lut Bw. Soc from de dom at wat 5103 (Apr 10) 1915. 32 DALLA FALL, GD Ucber das tomuntu wrun Klo patha um rouluptas betonthapu.com (Kepou) Kliniche und Pustologu lo balraste Arch. Data . Sypre, 109 3.87 1911 33. DE AVA), T Die verhanatore der haut Nunatuchs pralt Denat 25 309 (at) 1897 51 DE BS YD LLOPOLN, IC Idiopatlike oortugch uur om (Karov) A Du Chwa 3 1139 (lov ) 1938 55. DUARD CJ AND | Emkay FD Vultiple betrobupu vinum of Kapod Intolago stadies of to casa aux de boste intr let at mauropoy duck. Dermo Syphi U23 (Fh) 1025 DOUTLJ How of multiple lbopathic letartbag wiroma of Lapoul. Arch Den Suph 2004 (Out) 123 Doune JG apo wa inch Din Sypr 15 15 (fb) 1942 582 Dni 4 Wote w l wildle de Kupont Bu7 um fra une WC J +5 1934 Refaeiou 151 b Durant A: A propos do mochilation de la formulo mnou dary Tamporeticalo do Kapod Arch belon dond. aph, 0.100, 1050 5. Eun, M. A) OTROLA, AL. Kepods mrone vocated with Raboant tumor of the coloo. And Don Syph, 30.01. (July) 1044 Erg, L: 2nd fello woo corto beren. Arch. Klon Made 103:23, 1011 01 Emu OK, OS. Dle behandling dan salon idiopathicum heamcutaden (tentmat fabuchikab ploatam Kapod) mit QurKcht. Who worechs 63-2330 (Sept. 13) 1013. 2. E, A Ubat dac ball voo Keposturlom mut bauthorpertugor buldung und Wascotics Arch. Dorina u Syph. 16L650, 1831 03. LUA JJ AND KAT LIL Kepaula MTCOMO of the lap and lopom ulgaria of the not to the MD patient. Prosented at the Atlantic City Dermat Col. New York City (slar ) 1040. 64. Euth, F.A. Voltiple klopathie broonbapo marroma of Rapod. Report of cost to us Ammou Aopra drah. Dronet.o Syph. 30:700 (Mor) 1034 Eorex, L. As for diapon (Kepoul's sarcoma) ALL Arch Dermato Syph 13:400, 101L. 00. Ex L Cited by Gractwem and Couston (06) ESTRY V Kapoa 1 uro. Arch. Dernet. • Swph 45 085 (Dec ) 1913 68 Ewa J Cated by Frukt (816) b Ewa, ) Acoplexie Diaco, Ed. 1 Phaladelpbre Saunders, 1910 69. FU E 11 SITUT DEL AXD \ W.E. Kupon. urcoma davlatlag ws aken of the kep Staford V Dal.. 773 (0) 190 - FIT IL Sarcoon (Kaposl) Sock Dernet Syph. 18.011 (Out) 1923 71 IWDAY S Sarcina Kapood? An Der Syph. 21 003 (4px) 1913 a 11 (}ul) 1933 h FODAY S Kapod kartona Ibid. 30 m (Scpl) 1034 . FINDY с hapo multiple kllopuble bouchupour c. Tr Ray Soc Trop Vedo IIT. 351 (Dee) 19-10 1 Fyx C13 Кру Arck. Dermat & Suh i SJ (OE) 1933 b FrroR, CI fa Homo Schir da (10) 162 hapan's Sarcoma 74 FLD, JI Axo Covo DJLI StanlDO0 OCTc of hapods arcang, bland weddhabetes mellitus, Am I clo Path., 31.586 (June) 1851 78 Focher We, B.: Aleeplide und Geschoolbehlurt to Bethe Land Othon Handbuch der kunnen und parko logischen Physiologie mit Berucluchtigung der experimentallen Pharmloogle Belle, Springer 1087 vol. 14 PŁ L 1211 70 Foc. F Sure Stopathkum multiples haemornpaktom. Arch. Der e Syph, 119 10, 1016- Jhil, 137:107 1901 77 PLANE, F Chad by Bartuccini (210) 78. FORUCL, JA. Eaclothcome of the alta den lopty to the ser Home of lopto vulgar ao prourcome of the atto. Amin Sc 120-150 1800 the FOULAY Lapors Joena Brk Durma 17 171, 1035 b. FOOLAN L Kapodi conta. Ibid., 131 1830 c. Fouw L. Lapor, klopathie multiple plomout mitowa Ibid 57 354, 103.9 80m Fox, L tuon on 11 tee (205) b. Foz, IL ID (ILKualan on We F Lapor Hart Om Arch Denna, o Supt. 7.839 (Jupe) 1983 Bla. I u JF In dbc son on Traub (200) b. Then, JF Kepod's sarcome Asch. Danel Ó Syph. 1143 (Jaly) 1933 c Fria, JF In dexonion on Rubron (210) ALR. Murm, HJ zu Lesutstad und the rape (Anukeprol) der morbus Kupow. Derme! Il chrich 104 633, 1837 82 POT APA ak of Baltiple Idiopathk hroantunc urcuony (Kapu) Arch Dernd & Sypis 3153 (Fb) 1021 84 Pa I Clint wucha continuite of the ship Brw ] Dermal, 114 (tar) 1890, 85. CANO justolopo der Leithgolhaica Jure Genebrar derungen, in Drt Kralen Haut unter Berulahtise where Pattetowny w Wars Allowfo Be In Spraar 100,- p. 42 ou GAM HIC Kipor tanam bo lepro Arch Deto Syph 39 055 (D) 1938 r'a Garo TC Los col! NE ! por uncom Arct Dado Sypt &143 (July) 10.32 b. Caub TC AXD koho" L 1 Report of two (4 d bopathak berentaple neu (hapod) un prog mal features with special methods of treatment and losospiro Cua Du H 129 (June 1010 Referenca 153 84. Cum : & cute of Expert ooglome Cahiers mellomti de l'omon fro, 41, 1949. Aba.. Ecompte Wed, 3.85 1051 84. Go, WN. The retkolorem Brt.) Depth 58.107 1941 50. COLDKCHP Ueber wen fall von Arcore Idiopathicum bemostepena Kapod mt probnbichier wat diw. Dan. W daudhr. 100-104 (Feb) 1035 8L COLLALLI, HD AXD VALUTA, ILF. Sarooratada do Kapod. on med., 19 304, 1932 21. COUCOT AL. A BAKI, 1 Maladie do upod doma palm fro. BU Soc. frans, dental d wph, 15.357 (Mar) 1838 - COUCDOT , Buum, IL AND ELIALCHETTO Farme pls Dentaire et brandapique de sarcometom do Kepoel ibid. 0 1000, 1031 Garso 11 PITI, A AXD PETHAUD Acromercometro de Repod polymorpha bolcum formo atypqu. ibid. + 640, 1837 01. GLAUAN, TN Leopathic multiplo bomattagle coma (pou) report of Worstele to which the ful loan wu ontho e Arch Ophth, 1 ILAS (J) 10-12 B CALAM-LOS, EG Multiple klopathio hetontbaglo marco of Kapod. Brit.) Dind, 30.213, 1016, Carro, NY Elementos dinkco-eticbodloot do more do com fermedad (hromatoda) de Lapod. Som udd21 01 (Dtc. 34) 1030 od. CHOOTED, FUIL AND CONTIOX, AS Coolstol Hospida drum id Expow's wrom Am J V S 210.384 (OL) 1049 07 CAT E AND BOTTON, I Splenomegala emoce ed and adotelioma tanico tipo Lapod condone gume locul 1 ep onle Sindrome courpkna di ctiroloeodorlod perplauco-proplastica Arch. Im Blochun 4103, 1902. 3. Garconstw PS Zor Leontnts dos mlaplea kdorpathiacbeo hemontag bra wilony (Kapos) Arch Detra u Syph 14.384 19 + a. Carlo, Su di mo malo strunta del enocardia Patholic INOS, 1922 100. GRAD "Surtout de Report Dordo Abe, Librala y Editional 2 Ateco 1850 101. COMP Nattplu hopathic bernon bak harcoma (Repo) wuh ko - the pouth Amh Dan 6 Suph 30 183 (July) 1931 151 Rapal's Sarcoma CRO, P Lepool Arcome with low pude pidlo cell marcoon. Ibid. 13-188 (Pob) 1941 102 Gonowa, SL Cantnbution Tetode do lhbtofeocho do la pula do Kapod m de Dermed at rypt, 5:135, 183-L 101. COAXIAT, C: La cura del arcoma kliputico del Esposi con la rocotentcool. Anak ndah 2*), 1924. 101 GUCCI, F Contabato allo studlo patogenetico della Little di Lapod. Arch. cd. diad, lol. pol.. 41 1834 105. HALLI, A: Ein bestris ar kenant des hroma diopathicta moltiplet hacmantugicuan (Kaposl) Arch Derad . Sunt 79 5'3, 100-1. 108. IAM, II AND ROT IL Sur histologo de la wrcomatou do Kapool (Topenthelioratus wopathicum multiples av erposato Impeople to les cueneto) An dara pech 953, 1962 107 ILAND TOW PATLIDON JL. AD CASTILDA L LA Ink Adlc tutory Bru laut I 1103, 1912 108 HLAKKOR, CY Kapoaara cimal and ndiotherapeutic olien og troenty-thrco paticata Aeta meditor 31 157 (Oct ) 10-10 1094. JLAND VAY WA Case of pupok nted Droplason of the the J Coda • Tony Da I 01 (lon1883 b IANDAA WA Spontaneous in olution of uurt om. J Cdo CGL Dir 21 (Jup) 1640 110 Ilus IF FRYD, JL, Tmour JG Jlncis A AND ANSOY J IL Cathod ny treatment for hytoplom to ohlax shan. AVA Anth Dec 03 635 (Dec ) 195 111 Inou, IIB Indopachuc moltiple betonhapo watcom (Raport | Cute Duo 497 (ifer 1006 112 HA A En all courama Lopathkum olupke cutn pur port. Aber Arch Dernet - Syph 30.181, 1884 113. HIEY ILIL Jircon copolls and smiler (KILO to the Tego telo Det Sept 35 111 (Jap) 1917 114 Iloor, HX Sertama (Kapos! Uth Deens • Suph 1801 (Joh 1977 115. Ilenum F Surcou bxportugum multiplet diopathictum of Kapod ) Cotes Du 31 Lt 1913 Huang, U bet die bedrutung der brother pulboloock Nur II duchu ? ASI (Apr 9) ud -0 (Apr TO) 107 117 Tinu TF D kor RP aliquant bemendondotheliomu of the beat report of as I reh & Bet 1.311 1978 118 JLCC po tumar Dronys llung I 916- 1931 118 Rocronco 155 110. HODIAN HJ: Kepens worlome korting for your Arch. Dermo Sak, 18.957 (Dec.) 1926. b. Hacmax, W J. In dort Frukt (816) 110. Kocoux, O. Hypebemolyle of 100-plalo arigt Acta med cano 141147 1852 12L BOW, H Zor bletologle der Kuponechea Lanbett Corbal 1.7 Path u. path. Adet 63.141, 1931 11. Hubao, L. AND CALLAO FI La nousuno lopathiq pe Denduro multiple do Espodat o milerprowadau histopada uquet pathogere qact u dont d wph, 1-17 (Apr.) 1902 b. Hobao, L. CALLIAD PAD COL Paeudo-uretor de decoup do Kapal Bul. Soc. c. dermed at myph 35 380, 1028 13. NOKON, L AND Duront - Sarcamatan Włopathique de Kapud e lucen lymphoide But Soc freno de dermata yal 38.650, 1831. 111 H-RT WE. AND LOMCOLX, JL, CS. Stiplo banontegic mom uod diabetes metton Asch. Im Vad, H1738 (Mor) 1940. LIS. Jon, A. Santota ldiopatidicto hectarha patun (Kapod) Tord mar 12.3095 (lov 1) 1941 13 JANSELMI, E wp OTO A propos d'un ou do marcoma tono do Kapool: etode mubomoctobre et cual d'oculation aux Boka Bw. Sąc from do Detrit. a Swim 35:384 (Lay) 1024 137 JAD.S.DKpois carcoma de Condor 31.55 (Dec.) 1907 La Jopr C. AND LAUT ) Un cu de tonears paskelle ob word mo Cicoan. Bw for mech am 10:45, 192 1*JONDAY, A. Sarcoma Wopathiam soldpla basmantu glue (Depok A of a ctx precated at the fortrologiche dermatologiem be Gesellschaft zo lake) Hondtracht I praks. De 345, 1001 130 I m) Uber bertragung co wrote kiopathic to honor dugkwa upal nal brte i Drum 09118 1910 w Sarcoma opathie Capod, the, 15100, 10.00 131 KAUDUL B. and Mr J.F: Succo kleopathieta multiple bumontuoto of lupa, th perbal nounce lol ther to the South Vieu legro, and to reports Worth Unrm / S 11 (Vo) 100 12. LAN Vidpathabos stulpas pipiraterico der hun toch Doom wat tm 151 158 Kapon's Sarcoma k Karon, 31: Zug nomoclutur des Sciopathbheo pigmentutan pod. Ibid 29:164, 10:44 c. LAPOSI Sarcom, multiple pigmentosum. Ibul, 3.133, 1800. 133 KOL, LA ar of Sapca's hectumpanome Arch. Der Childhood 37 153 (Ap) 1952 13-La Luncur J Butiple herkenbago marcome I Can Di 31.573 (Aug.) 1913. b. Locum ) In dous don do Lane (141) 135. LLARLA, A Case for din nous crokratiche chronka truple KA? (lator pared to be Lepod's topathlo houpute urtom.) Bn I DATA, 1934 b LLALLA, R. Kapow , hotsa Ibid 50:37 1938 WALA, Rhapoos Uhren. Proc Roy Soc. Vad (Sac Der wat) 30.010 (Alay 20) 1962 KOCHRO, E Kapou surcuna Calarase boy (and 18 ) with low albzalon on the left wir estremity and on the right crunula lecomalu Dermatologar $ ), 1910 137 Koux H Zu Lenntnis des alguna coronarose and dat butun om besondere Art i Danmal. Wypoh 1 300 1603 v Vorste Duas or los 00 multipko budeurhon de catreo laten Bert No. 1 hech 1813 1856 blon, 11 Kronleonore Dung (diopathicha mutapis wr coma bacoontaget der extremblaten) Arch Dermat Syph .3 119, 1891 138 L1, C Veber pamome Arch Dermet Swish 41.1 1009 132 KAYO Sarom Idaopathicum Ikmorthegou um (hapon m JADUSLI llandbuch der II w wad Ceuldevant Aedes Berlo, J Sprepr 1833 L 12 pt 3. p. 311 10. PRIROW DP Z fnpt des mullglen kiipathische heroin rtwas bro Aruna Lupo Tantra llau. Crkh 011" 19.03 ktu , су Benicom ind malignant tomon of the foot LAVAL 01 (Mar 18) 194 1-12 Luzon HY A no of wropone moldplex klaputheum (Kepol) ith fatal Outrete. Uret • Cita Reo 37 230 (Ape) 1933 1-13 LANL CG AD CuN100 All Lymphoblastoma (COM fungoid) od bonbar warume of Kapol a the wind persoo Arch Dermat i Suph 6.13 (Arr ) 191 IHL LAVLJE pou wo such Dernet to Syph 26.33 102 141 Reference 157 145. Lux, PJ AND RARE L Veber du nufunung der Kapo Hluchan lumbat als gestato anglocator. Zirohr Arbe forch, 1269, 1635 14. Larona BLopathlo multiplo pigm.cat nicom (Kapod) Arch, Dond. • Syph, 33:10 (Jan) 1938. R: lohipien Whopathdacha bemond placben bauturkan. Asch. Detay - Syph, T0:14, 1004 14. Looto, V Coopdeuren e com a utoma diopatico fi Kapod (gloendotelioma cutanto di Espod) di pod com mo tudo ad nolu toc Archa tel. donnet 11401 1035 149 Lun, V4 In deciso on Suobbo (141) 150. LEA W.FGtopatholog of the Shu. Ptaladelphte, Lhopincott, 101 R 378. 1314 LITUAL D.: Sarcowntowu cutie JALAN 39:1434 (Deco) 1001 LOROTIAL D: Idiopathlo multiple hemorago Micona (Kapool) Trtume an eological factor (?) 1 Cten. Du, 77.822 (or ) 1000 152. LOVDAL, LA wktplo Scopathlo bemonbeglo mrcome of Kaporal (report of a fourth to a blooded Negro) Arch. Dend. • Srph, 1972 (Jumo) 1838 153. AK CANTAT ID AND PACE, GT Habpant blood vend tumors. Swi. G. Obat, 01 488 (Oct.) 1950 LSI Macay, JT RICCA, JJ AND CUMIX JF Kepod's WTCOMA following alkugla upit Anm. Int. Nach 42221 (Apr ) 19. 18. MICKLE, CI X Rated Redion the Trecut of Dir of the skin EL 2, Pulderble, Lan, 1077 P 707 tu Alachu, GIL AND COPOLD, A.C. Idiopathic multiple bor tape merou (Keped) Am. I Cancer M.1 1938. 159 MACKD, S, Raverks on roos den of the thio Brt. VII (Jan 4) 1890 ILUX, S. A cow of Idiopathic berobage ercoma of Ispod Am I Die Ch., 1 137 (\lay) 121 138 Var Lion J VIL lots the hitopathology of miple opathic hemanthape toca B. ) Dec 17 173 (Hry) 1905. 150 Wax TLAND, F Carl Ltrog (110) 100 VAAL G Bahnastia Kapral betoodan borudakb tummy dat perak blakatlonca Arch & Dermat. . Syn 29 y 199 und Ueber des prom klopathicon Kapak Ibu 34, 1000 157 158 koposi's sarcoma 101. Sannox ) 4D Homu L Immo beoolytlo nama la Kepos I Marconia hybcenl malcment only Arta med duro, 150-3, 1854 102 LALOx, SLP: In decuacha of Perod (203) 183. LAHOX P A nerd pigmentares, tomcws Derrouch da dod path 3 417 (May) 1928 and 3 857 (July) 1028. 16L.. Siamo Cuted by Darlid (58) bunior LL AND BLOOL, IL Tadbook of Latolog EL 3 Philadelphia, Semdet, 1938, p. 83. 185. VODO DA CONTA, S., Ein fa] ron Kaport's plamcorhom Festuchnut fur Unne, lo. I Dont Stud., 20:112, 1010. 168. SINAL GL Sarunu mclanopi goncatosoma multipkt cula. Vondtacher pralt Date 30 516, 1900. 167 Vera DS. AND JACOBOY, 1C Voltiplo honourhage or Cow of Kapool Am I Pak23.1 (July) 1027 163. VIHDOX, HE. La discount on itchell, JUL Wycow fum cordes trated with sodium parseminobenzake wcd duch Derme Syph 00 885 (\or ) 1019 10 ou, H Sarcoma wblopathicum multiplex Kapoule Arch Den # Smart 185.57 1012 10 ar HE In dictados on Imbel (171) 171 Vrem, DS pour urcame doch Dermal. Syph HI 801 (Fb) 19.40 12 VULARD FL, LA Krpar wton with secondary mvolvement of the lefanum, perforation and peritontti Lax Ind Wed, 31 324 (AW) 1919 13 LOCO I Ln doudoo O Tomy (259) 174 JL Kupov s srcune. Arch. Derme Syphy Os (Apr ) 10-13. 175. Vul 15 Uber polypore boartis, metastatode couchabandh on how mbwatig throunbeu ck kulcohernoon Itchor Arch path And 24 105, 103 190 CHITT S., Vans, PFA ZINAY, HV DAJ um klapathie hemorluk whome (Kapourdhe report of a cose th Decropsy Badma de In Val 23 001 (Apr ) 1915. VAX BLA Multiple Wlopathi hoonhape (Raporl) productog urethral #rkture Arch Dernet • Syihin 1-293 (F. 1913 18 KILAS AND FA L L A propos de l'interpretatie ik lele too ste urmatoset langetako pipocotate de Kapor Bd Sne fra de Jorna sypt 33 15, 100%. Хан HD Kupost leporthal warcoma Art Direto Srph 22.913 (Way) 1930. 13 lieferova 150 NUTO 180 Moo F. Zor bistop der toglouto's Kapod. Dort Tichert 191.267 1900 181 Noron, V: Afymoon of the hurt umlatex broncha pa AALI 11 564 158.699 (hor) 1819. 181. dora Plania & Keponi ed aleatod obal Chor Hadle di Ottel, 3204, (lay-June) 1960. 18. Oon, 3.7: Sarcoma (Kepad) Asaka Dermat. b Sypt, 18 630 (Oct.) 1998 18. Luar Putri In discudono Sezou od Wie (132 b} 16. Cyr, S. patele buting pagsusted (Espout) Arch Dermat, Sypin, 51 535 (AUS) 1969. Indha One OS 40 JITCHAL, J. Idropulac multiple beraar thuck wrcome. Arch. Derrot Syph 3.47 (Apr ) mai & Oman OS. AND A SATILOVAT H Dimes of the show. Ed a Muldelphia, Lom, 1843. 187 Oson, EA, JORDAN IW Houx, PCAD Ram, F3 Narog mostart thermy Barcou Hartanatoo dumthon AUA 3.61183 (Dec. 57) 18,47 1884. PACL, CT idiopathur moltiple bevonbegic Witome of Capoul V sor, Nons esoy 34 803 (Oct ) 1937 b. PMI, GT Tumors of the lands and fas. St Love, Houty 1939 P10 100 PAOLn, R Sol mnogu moltepine promutiro Kapod com pounk nruanto allo bocalara whacerats Studio atako Inatomeisto-potopco bon trebalco. Tortabl 1 a * Com 28 BIS, IS 10. PANO Camisa Sartoon het onherkco de Kupou, relacion de ID nuo wchodno de la non do color del loc. how de dent. 2.100 1931 101. ParTAN, LA AXO Douth Sur low lesion naulo Dervente de u pouco de hupou Sul Soc from the same a wypt 35 145 1928 6 PALTRO, L. AXO DIH, A Kepow CONTAO gore To but Durial proexta Dr Derne, 12-63 Partun LVA Asglosassoo Kapont of the becam memb s* and we shoulemy A od treated at tils #th t-tay therapy Dermatologia, # 100 fra CT Holople bemantle po uno of the past the Stec V 32. 137 (Frb) 1970 in dry SDO O W (387) 100 Kapod's Sarcoma 19L POLADATTI, IL: Dre botuar komutare. Vordih i prill Dernet, 35:119 (Sept) 1802 and Ueber dus hrom multiple Espoo Arch. Denne. * Syph, 83-378, 1907 1852. Pour L. Del Sercom ctor Calanos. Paris, G. Stathell, 1890. b Pun L. Surtonnen la L Pratique Dentelorlans. Parts, Jlagon 1904, vol. 8 204 198. PT BP AND LOA JA Multiple Sclopathie bomoabe power coma of Lapowd in 1 ful-blooded Acero drol Detodo Suph, Uro (Ape) 101 107 POLO L Ueber das han come Idiopathicum coth Spor em bottes mor urcomlehra Vichoc Arch f puth deal. 107 54, 1902 1984 P, W Zur Leontnts des Lapon xhen pago cotturhan Arct. Dermat. w. Syph., 8.007 1007 b. PT W: Zur Lenntnis dan soul angoms und remar berkebunon Lum Loclocholuinz Ibtd 99-109 1910 1002 POLLITIO, S. Cited by Pauthor and Dix (1016) b. Poum, S. In dicto co ltal (27) 200. POLOPUL, A. AND CUTALOVA, IL Zur frage den puoluples who opathbchen bacmouhaginches warkom Kepon LouL HodCeach28:171 1829 201 Pura L Betris ar Lrantau dar hauturhalde D. chaxhr. 91 1815, 1830 and Ueber do klopachache mul tiple pigmensburloor der baut (Kapod) Archi Dernetu Shph, 16L167 1931 20. FADAL F Contributo Il conou ria del Arco nattplo all oputko corrinco del cutr Chor the mal law co puta +3 133, 1002, abstribud 16 97- 1905, ad Sal proxeo AD tam-putolopadl woont diopatico di esposy (ango-ndo telome cuteneo di Rapod) Glor med dermed 71 1501 1830 203 FLAVU, E. Sarcoatow dlopathique corupur (Kapod) BU Soc fex de Dana d Suph 31 557 1020 101 Ranhu, IL AD LILLI Kspou nou. Arch Datud 6 Srph 2.514 (Sept.) 1080 RAVAUT PAND COM, RA Ge of Kapu's cute cow w coot uixoCualed with autres of the lone tra md . purpurle yadro Abstr: Am J Carry 10.1317 192 200 RA Primary tumor of right auriche of het rupture of thletar du av Brut 21383 (Oct 29) 16.03 90 Rrer Sanctool cs diopacke Arch Derma Syphen 0 13L 101, DJ Eln thoarderool coda (2coa humor rapuheo hautuarioro bal *81 1903 Reference 161 3. Roca, 1 AND VALP Comprado nedaleko at estrito throw tytique de Hecho dubio e conta com tot de Esporl Bro Mohe 65 1467 1873. m. Rooma thes Lepod's woporatowe in Negro of Bagles Congo. Arch. bolpa Del * Spo, 6:18 (10.) 1050 1104 Ranchor, P AND LOR, AL Lepants arou (poreticulo toda) Poprad u 14101 (ADL) 1953. RockAE, AND AB Bare lotions in Laport's Co. Aua Arch. Deras Qan (Sept.) 1851 BCS, VW B, AB Lycoplanposuus tussyor ta Igoule screen, Proper rud a monitory of de Derman Surat Baaben Calf Impr 1850 to be pobed to ALLA Arch. Dort ZIL. RA. Veber du att prat der hebat taler normala ad pathologischen bodengungo Gorllect Morph w. hat In Hauchen. Spaberidde H17 1006. 112 Rountana, A. Atam for din goosh (Keports sarcoma!) Arch DowSyitte 2400 (Oct) 1931 212 Ronnu S AND ILOCO, AB Laprelmercomra. Anch Dermet, SupotH.400 (Oct.) 1963. 274. ROTTLAN KG Zut kepritous des we opattumn orld plex hentais pomm (Xapod? Arch Derma Sth. 154 134 (Dec.) 1937 11. Routacar, AC en kann, Kepou decu Brut. I Dermt, 30.375, LOU 210. POLNOM RK Multiple today the stana (Kepoel) arch Dernet & Synth 19.100 (July 1834 317 Sarpu. V WA ND AND Cir | \fun phr hemorrhagle was or uport In Dewet, 8.317 h Sarti, W AND CATV Lapos KODS and lymphatic keo Letole, report of ach bietologe act of the two darura in the same brian anh. Derunt & Syph 5125 1 Viny) 133 16 SATTON, C Carbala alle sinho Hoia kopathicum moltplan harmonterima Kapan Gior to scruet af 373. 1833 9 SAM 3 24 hrann der sarcoma Wipeikino mulupky harmontuakuan Kepa Arch. Dermat a. Syah 114.071 7. SAS TIN DL Curt the locker and Cparano (1561 h S TISTIN DL to donio a Crox (1015) SF CAD 3 M, H G Kupov war wch Derma Syph 38 318 (Arg) 1932 162 Kaposi's Serconta Satu LG 1 The Blood Picture and its Cliek sipore St Loul, Souby 1928 23. Schov, E AND TXCDOTA, : E. fall voa kiopt. thlchem roultiplem bacinortlagbacbem hautuartoon Kupod and deput Rontgenbehandlung Dating I ducher JL OS, 1832 24 SCAD, CG. AD B20 Li Em sarcoma Klipathicum moltiplas hemorrhaglown (lapod) mit barnetstest. Arch Wb . arper Dentalapla 201.09, 10X. 225. SODEDOT O.EL Espoof's Harbome with toyok cent of the aropharynus and Lyn Arch. Denne Ó Sypin, 15X133 (Feb 1012 220. SCHOLTZ, W Hluluples kiopathbuba hantarboo (pool) Arch. Ditu. Supoh, 31.300 (Nor) 1680 277 SCU DOIR, EL Sarcoma doputhucum makitplaa cutie. Aber Vond nach prale. Der 0-0, 1808. SLADDAL, KIL Kypost's the report of heathma) beerel mamftaboo Radiology 51 248 (AL) 10-18 29 SILIKOOT S.B. AND POLAN, WLE. Ele fall on mercoora do puthucum multiple bacmontupom Kapool. Arch. Derinal W. Srph...331900 330. Sau, ) Wotan bertrap tur patholopo do moy. Sarcina maltipla Pellucmanh Idiopathxum (Kapo) Arch. Der Sypilo 68.1 1903 231 SOLLOW TU Zabu fede des Aroma Kleopathkum poor tom maltiplex Vanslochr palt. Dorne 95 839 1607 22. Spon, FL AND CUNO, AL.R ultiple kllopathoc bemorluple Arcome (Capoul Arch. Dennd. Syph 35.17 (Aug ) 103.8. b So, FE AND Wire US Vultrpik Idropathie hoorham LUTOAN (Kepost) Ibu 27 851 (Slay) 1833 13. Septmu, J.JL Care of Lopathie muliple plomeut unum (?) Bm I Derud 13 901 (Jue) 1901 b. SOQUDRA JH Staplo bogathc pupment warcowa (CD) of Kapou, with riport of no rly cunIbid 5.331 (low) 1913 c Sem, JIL Socled multiple loputhe powo wo (Kapo) Tbl 33 377 1021 d SLODU. JII AD Bury, RLT Descopera o true who the case of malople whopathic piment so-called urtoma (Kaposi) Ib 35 50 19% Roferesca 183 OD 231. Smau, Gi Contnbuto clinico od wtopatologieo alo Rodo dal condotto wrcome cuteneo Wloputkeo multiple apo plco di Kapou. Donofilo profo 0.653, 1931, Aber Am J Cance 10.1007 1838 133. SHOXTO, S.A. Pitmary tumon of the bourth special do enco to cartum fator which led to abgeal and correct dogs before death. Ann I Had, 8.340 (Sept.) 1035 ST, B. AXD Condon, H: Iqos mau Carrput with unique vicen munostations. An Sury, 135:118 (Jm.) 1022 SILI W.X A of so-called "Dubih Hemontagio Sar (Kupod) Bat I Dernet 32 331 (Nor) 1900 338 Sara, H. Lepod's NICU wah matrament of the opArch Donncho Syp 4711 (Oct.) 1041 3. Sav, 8.H. Multiplo bopatho bamontago Deon Anch Derud. Syph, * 60% (Sept ) 1041 So. 11:4, SH Expos marcoma of Afteen you dorton, Iorche A06S (Dec ) 1043. 240. SO, EF Notes on of multiple pomcated wdopathlo Com (Kapool) Br J Dernet, 31-18 (Jan.) 1010 24. BULA, 1 Multiple kopathic bacteunhanc puted room (Capod) Brih Dernet, 602.383 (Nor) 19-18. 24% SMIT, D.8. Neoplertso alredeat of the boat JAMA 100 1102 (Oct. O) 1937 24. SMITH, HJ AKD SALOTT, MH Multiple xllopathdc bemoghadlo Bu of Kupo Arch Dome 6 Syph 01 (July 10.10 241 Sao P Hooghegic Mirton of Kapod to Negro. A Arch. Denn 72-501 (Dec ) 1885. 245. Srpen, GJ Hotple pipooned bonoa bagie mring of kn pool arch. Denat & Suph, 3074 (Nor) 1994 STATI D. oktyal mumftaboon of Empor NCIO.JHL und Blasp 19 071 (Jan. Feb) 10.11 2-17 810,1 thpresuarlame der freiteten Bert Lint Hekuur 13.634 1808. 28 Sans, C Veber de ma mola hecnorthagkeun (lapon) Anch Dudu. Srph 111 331 1012 10 Sயா FIV AND THEN Lyopher o port etclony hepbeden Cover 104 (My) 1044 50. STILLI. All AYD Sury, I EL por o with huo of the im Arch Deind. & Sypk 4500 (lar) 1940 161 hapod's Sarcoma 31 Snn2, LH, Eno, HB, HondOW XL AD Lomwe Lut Cytot ogy of Lapods arcome. Arch Dernet • Suph 67 (J) 1053 252 STATTOX EK Slobplo hemoabago atome of Kapou. Orol Cwton Rot 3.71 (Jan) 1929 953. Suzyce VLA Multipto Kleopathio bemahaglo unoma (Kaposl) Andi Derudo Syph., 30.455 (AUE) 10J1 25-4 S1204D, D Laporta diem Arch. Pat, 34 61 (Nor) 1011 b Snow, D: Personal cumununautton to Perly and Line (100) 255 Syxor HII AXD KTE, S Krpool's disease, report of a caso wab unosu oropharyngeal mascotations occuning to a fubblooded Nepro. On Sun, 1337 1931 2.58. TALBOTT J.Il Cimaal mamfestations of Hodgkan disema New York Slate J V 1883 (Sept. 1) 1917 TEDOK TIL, CG FOLSONG UP AND CATRU, TJ heral hapoor's drons del Pdth 13 335 (Apr ) 10-17 258 Toroc F Idiopathes poultoho barorrhaglo prome (Kapod) Arch Der o Supl 25754 (Apr ) 1932 230 TRAVAYTAM, AD FITTIPALDXC Coolnboto lo vodo do mma gloendotdowa cutaneo ckl Keponi ed all newstenza de chiferente Blastomi Arct itel der W 7-215, 1931 SAD Tul Er Sarcom.t-spot Arch Dermat (Sret 18 (FH) 184 201 Tur 16 In Lunetuloa on The (285) CraviYE Bertrag nur trupe des cartom klopathie mult pics herontapetuo epoul. Arch D « Syah 150 120 1920 903. UNA, PG Istopathologie de llaut Kranklin, 10 Onth, I Lotuhoch IT Speux Le Photo Apalok Bodin, A trebwald, 1604 Eschungband, part 1 9841 U HUO G ZAVIOLI, E \PAOLO 11 En vob Lunk be wowe in mco do morbo Kapor Glor dow med Torte 101 101 1835 205 YILJIA Dlour CA Case of Bopathic Armor the purua (Upa) with colopry find Calon Reo 30 348 (Apr ) 1035. 2002 L P Va poucano de sarcamatak Lepost. - Sor tror de dermed of hp 43 100m LICNS P AND PTT Serra diopathieueck Kapow in 19 yo 143. loco, u full so hochg.digt ophilscher pik bu ok urheu ma poa Arh Dernet Srpt I SL, 1901 na RsferCROA 165 Son WALLHADO, JIF: Bichlid and mawry to tho tromet of Hopathie multiple bempatague wintomt 1. 53.1008 (nor 13) 1009. Dobe. YALID, : In disco on Bilvoo (239) h WALD, A In durcmpon da Silva (1396) ra WAITEIX, AS, The popote Dooplastic relatamhip of Hodylos dren, alevlook and lool.coube hymphoblastoma, md mycords fumgold sna Sun, 23.153 (Jm.) 1931. 171 WATTON W.L. AXD IcCann VD Blood and lymph vem tumou A report of 1,058 cewe, Grac. Obet., 71.589 (Nor) 1940 172 WAY S.C. And Klon, H. Haothomlberton md puter from. Dernet. Zach :139, 1920 773. Wan, FP Hemoulaglo trianglectus of the Onko typ tulanglocatie dysplooi An nolated cun, with duncedo ou maltiplo poloaung stellato telenglocta ad other riktig hoemanglectatto coactions Brut. Derud, 18:182 (Apr ) 18:30 b. Woo, F.P AND Dam, P Idiopathic multiple pige mcuma (xdaopathio multiple hemorrhaglo ricom) of Capod Ibiche 17 133 (Apr ) 1903. 174 voto, JR Sarca klapalbicom multiplex berwcarbagerom (Kepon): nurkadloos from the noul chodaal picton Anch Det & Srph, 30 383 (Sept) 103-1 178. Vein, GH The moul upect of audiec borotv.ont (debt mulr tumor) te klipathic becocheglo watcome (Lapore disewe) A D Wed 14314 (AL) 1940, 78. VOD, GW Act of valtuple chopathe pigmented arcoma J Cuten. Di, 16-205 (Way) 1802 பy | AAD Sபாயா IL Intostmal dan LCMC eod tatrabdontol begloendothebo moc to pattog methyl holanthroue. J Canart out., 3.331 (Dec ) 1941 r. Vart JEU Kaposi's Wedpathie Mit Prec Ro Sox V 30-227 (Der D) 1915 ed Idiopathoc beindarlage arcana apoul ibed, 0017 (1lcy90) 1013 T 11w UJ Lo diction an Orusty tod Mubel (1801) 280. W RA The Pathology of Ton St. Louis, louby 1918 p. 60 1 llo FA Clime o refractory court het falat of relainety short Juntoo, corporis, pontmartra todop (port muy fibrosarcome of the rate rundet Prec Stet licct. Vgs Cle 13 331 1838 INTENTA Vulturple herboghagbachu plamenko Ke pod Louifilm u Cack -#161 1077 188 Kaposi's SarcoRLS 24. War, LHSlycow fuo poble benigo wd malignant coll dysplass Arck. Denne • Syph, 50 450 (OL) 1917 lomouc, M. ad llooun CV Jei Mitropitoxand that apy for flodglas dueso, lymphoercoure, the kulemus, und a disl Cr 1357 1948 285. War, F Idlopathlo hemoubuple multiple urcome of tbo alto (Repost) with modal dacco to warty demonft. Rec 88.813, 101% blour F Sarcoma of Lepaal Archa. Deras & Supt. 18.042 (Doc ) 1028 c. W, F Sarcoma of Kepost with bocal ladon Ibud 33 (y) 1911 d. Was F Valdiplo klopathic bemorth ple aroom of Capod kth while changes to ly corpb pode Ibid., 31.291 (Ap) 1045. . War FuD ELD, JJ Ballons crapuron lo hemontugle or com of Kupon betn plasm. Ibtd * 625 (Slay) 1933. 280. Woon FC. Cated by Hlackro uod Cipollaro (155b) 287 Nou C Idiopathic poluple bemontage ATCOM1 of Ripost Arch Dame & Smart, 36 1252 (Dec ) 1937 298. Wour In chectetudo on ROKI, I Kaposd's wrcom issociated wth lymphabc kolom Arch. Dermet & Supt. +5 506 (\ov ) 19-13. 280. ZALU, SJ Leporer mercome Anch. Derma & Sypt 4.19 (July) 10.50 ZABOY, SJ Idiopatha multiplo bemorrtu pie sarcoma of Kapod Ibod 55 01 (Apr ) 1967 © ZALOY SJ posi's sarcoma Ith moh cent of the castromtrttal tract. d 57 75 (1) 1848 20 Zabun EP lo dicunton on Cornblert (19) 991 ZUHALAL L: Vultiples thropathaches bouturlop mu laochemetastce. Arch De W. Shih 107 329 1911 INDEX Autobathoooo apon, 35 Abdoute pare, 106 ALO corporal, 81 Authod os, 130 B Achadon Schoenketai, 37 Acrociatatit chronios Atrophila Hudder, 102, 110 UN 131 Blastotytona, 38 Advost gleads, lot Hledin 70 Andreas involvedumat, 123 Dlood rok, 0,02 Akoba, 30 Blood rekel, ocuplete, Allergie uterus, 51 Borse savotresel, 114, 117 118 A, woodery u 100 Asaris, hemolytie, Ina, 101 Ba mano H, 100, 101 Ampoblast, Bruka, 103 Arrozbrotta, X, 63 Broed kort est, 183 Articol, 130 Bollow beton, 2, 3 Appoma, 38, 39, 40, 41, 1 133 Surotoy TO Adpowtants uberk, 4 C Angrohad, 12, 344 33.84 All pool, M Cmpadarias, 40, 66 67 Arrctus dia 17 3 Crollery backle Anomaltapy 1-45 Capillary direttoa, sa Asspbesantre, to Charorea, 50 Artemom. Koul, 34 Carenon, 197 Alvaro Contou bemangkale, A Asochied with other han, Colesta, 30 120 Centre De Tous systra, 12 WOO, 197 Choke themars 145 duabete Dritan 10 Chuldam, Areso in 8 buckwear Catation 4 Iloupec Jeak loud Clubbers Aek, 42 huphux bic Irolomu, 125 Coll, 19, 31, 30 hupkan 12 Conuest, 35 hrapbourne IL 125 Coutta tha, 3.8L 68 lupte valparnis Cutis 32 to fuokles, 2 Cruis, atk carstk kua 1 Sao Parabrogtunkas horase * D'Esbitro ope 83 loopir Dubeth at L, 193 167 189 Kapotti Sarma Danhou, 108 Dla grade 130 Depoule, diferental130 Dog xcmith sty Dysplatts, developeemt, al E Geographical distribution, o Cheat colla, 37 & Guttoforeta, H4 40, 64 Glomos tumor so, a, 130, 133, 1344 Gluteal orahe, 31 Grupul bod tur 58, 68 Granulocytopenie, 101 Crunnlamı, 37 50, 63 Crabalone poponicum, 10, 03. 130 133, 135 Cronaburetot stage 50 11 Eu involvement, 22 Catu, 40, 31 Edema 73, 80, 83, 89 Elantre ore 01 03 Fkplentisks, 75, 83, 87 Flephandru, lymphan plectati 75 Endothchual elementi, 31, 35, 39 15, 22, 68, 69 70 Eunophilie, 100 Epic, 152 Epithelona, 09 Eryuupelan, 30 Enthrocyt Ebooty + 8 ko od sex, 7 Occurrence in the younL frapuntcal dhanbation, 9 pred potong factors, 13 At of prechleston, 1 E obabago, 08 Eith tution of Woul, 77 E truths 18 E) 122 123 E), 97 Ilhas, 13 Ikealot. spontanco, 34 II at, 97 104, 105, 119 120 1. Ilmaturu. 89 110 Ileapopram, 99 Ilemenstag, 52, 54, 70 7 60, Da, 108, 139 Hemorrhagic ff non, 103 IL Kouderia 03 ILudmith, 118 Hkto te 7 Incarica 3 Ilodgla hot 23 124 Hydrocarbons, 31 I F Fact in cincot, 02 Fuhrm, 68 Fulroup poma, 62 Fibroblast. 18. BL Fuplus hwors, 98, 99 Fungos bolag 37 Loxx car 7 Sindows Annon, 30 Lafectou Luty 25 Infutrin 518 Ldamentory Hart 51, d.o. Internal or bomoh tu, 102 luster, 07 103 Ido merobon 111 lo cluinn, wens of 07 08 Inco Not 5 Italunko с Castrointed txt 102, 103, Inda 189 Lipe, as I ole Oban, og Modes, 102, 103, 119 Klinca, 97 104, 105, 116 Vyroda fumpolde, 43 183, 130 132 L N Layo, 29 113 Lattice fhart, H, 18, 04 Noli Jubble 42 Leposy 130, 132 Nuk leukocylke, os Lech.com, lymphocytke, 43, 195 loopbarys, 13 Lailmle, molocyth, 43, 1. hectads 64 Leukocytools, 89 Acto pare, OCCUTEC m, 10 Lauhorychle, 02 Mooplestie sapo, 50 Lxbce plannen, 50, 130, 131, 134, Acoplute theory 34 135 Nem, datod from, 41 70 Lerrous mitti buvolvement, 102 Lirer 07 102, 114 Neurom your or gloro, 17 Lamp, 07 102, 103, 113 Nodula, 46 00, 70 Lupus vulgus is Macula, ulceration of, 83, 85 Lymph, blochers, 78, 88 Lyop, pullarken, 54 o Lymph nodes, 07 102, 103, 100 101 100 Oarpation, 18 Lymph, tula, 83, 83 Oral, 2.0 Lymph, nucular system, 34 L mph, cicha, 03 Р Lycophocleopathy 06, 07 L mphade vi en Pon 70 Lropbrkme, 13 Palate 20, 21, 20 Lauphocytold tk, +6 +8,03 Pancre, 102 Lyrphome, 123 Pathograd, 2 Lymph narcom, 13, 4,1 125 Pathology 34 mabd netowy 31 pathogenetia MI neoplastic theory 34 Luk 522 infectious theory 35 Vaculopapolir lestoon 50 malo vincular dute, 30 Vucl, 6.00 dorned from one time. 41 Welamin, 00 Articulacodoch al n drohr- Tutek kom, 105, 130 perpla, 43 Vicotx Brutal OS albot tbroken . Woovy tak, 40 Peak 31 07 103, 110 Matild analoony 31 Pentheblach 34 33 Vucut abrur Rocca, 10 1 - 170 Kapod's Sorcomo Partoofth, 111, 113 Puery AL, 20, 100 Pamet, 46, 52,06 Pigmentation, Luck of, 01 Planeetation, totul 88 Pitotary gland, 102 Plato, 72, 82, 83, 85, 88 Plasma arh, 49 5, 02 00 Platad-like formation, 10 Pleur, 113 Polyuter tha nodou, 41 Polymorphism of colk, 63, 64 Polyvascultis, 41 Predispontng factors, 13 Accugabog, 15 trama, 18 Coll, 10 vakuler changes, 16 arcuks, 17 Propous, 134, 1-40 Pruntus, a, 25 Purpura, a, 54, 72 TD, 77 80 Schwand cels, 41 Scharndombo chungas, 87 4903 Scrutin, 32, 116 So, 7 So bormone 51 Sites of prechlacton, 17 attinther, 18 walp, foco and aun, 12 anal, 20 Skin color 90 Sptada cek, 13, 63, 67 09 Splet, 102, 114, 115 Spkepomerely Spontado rep, Starts detroaktis, 85, 80 Stewart Trma yodroos, 13 Stodos lostbration 16 Stounech 100 Stoputath, 80 Surgoal therapy 143 Symptoms, 9, 10% Rubferttre, 70, 109 obke tive 72 Synonym, 4 Syphalı, 30 130 131 133 Systemio suscular dbete 30 R T RACH Incklence 9 Radomo therapy 140 141 ToontoothTypy 140 radium, 1-12 catbodo m. 143 Raptary tract. 113 Fotoepilobebulan, 43, 44, 64 Racecou, 13+ Round arth, 30 $ Tetanglectave 40,8,01 Teta, 102 Thrombophlets, 89 Thyrond, 103 Tongrie 01 102 Toont, 8, 19 Trxbe, 114 Truma, 15, 30 Trotineat 37 relation therapy 733 tra fokt light, 39 Atpul, 39 bembat, 3 Arbor 39 Truphle dostalace 41, 91 Serend, 130 131 Sucatne, 3L 10, 9, 54, 43, 97 IJ Salp ince kod cars, 22 Scurs, depressed, as Shambres dkeure,
Indor
171
Tuberculod, 113
Toon, 02
Tekik, hmph, 83
Vird, 3
Von Redboghants Iber, 2018
U
VY
Ulcention,
Ulomon, Do, 85
Utervloket bipht thengy 143
Umgeodal tact, 115
Vagner le moc bodia, 11, 12
X
V
oderne placborum, 130
Y
onkodila, 48,88
Teoler ches, 10, 54
wouder the conne, 30
Tonicoon lenioos, 12
Young Courra ta,