Vascular Tumors of the Uterus

VASCULAR TUMORS OF THE UTERUS PAUL

II. Malignant Vascular Tumors PEDOWITZ, M.D., F.A.C.S., BROOKLYN, N.Y., LAURENCE B. FEI,MUS, M.D., F'.A.C.S., NEw RocHELLE, N. Y., AND DAVID M. GRAYZEL, M.D., BROOKLYN, N. Y.

(From the Department of Obstetrics and Gynecology, State University of New York at New York City, College of Medicine, and the' Departments of Obstetrics, Gyneeology, and Pathology, Jewish Hospital of Brooklyn)

A REVIEW of the literature we found that most reported cases of maligINnant vascular tumors of the uterus were published before 1920, and, because of the multiplicity of names used, as well as a lack of correlation of isolated case reports, a great deal of confusion exists regarding them. New histogenetic concepts and diagnostic criteria have necessitated reclassification of all vascular tumors of the uterus.U A comprehensive clinicopathologic study of vascular tumors of the uterus was undertaken in order to clarify this group of neoplasms. Benign vascular tumors were the subject of the preeeding article, Part I of the review. This section on malignant vascular tumors is based on 10 cases: 7 of hemangiopericytoma, of which 4 were previously published,' 2 and 3 of hemangioendothelioma, obtained from a review and reclassification of all cases previously diagnosed sarcoma* or hemangioendothelioma of the uterus. Angiosarcoma is the generic term applied to a malignant vascular neoplasm and includes several varieties of new growth based upon the cells of origin. An angiosarcoma is defined as a highly vascular tumor derived from mesenchymal angiomatous elements, which reproduces blood and lymph channels varying in the degree of maturity, and which may be occult or apparent. Angiosarcomas must be distinguished from some highly anaplastic sarcomas wherein groups of tumor cells may be noted arranged around vascular channels, particularly in areas of necrosis. Such tumors are designated as vascular sareoma, indicating the presence of a rich blood supply. In addition, differentiation must be made from other rapidly growing neoplasms which may also have a rich blood supply. This is especially true for mesenchymal tumors, which are derived from the same primitive tissue as blood vessels. Thus, increased blood vessels within a new growth do not necessarily indicate a vascular origin of the tumor; only the presence of vessels in various stages of development and maturity points to an angioblastic derivation. Intervascular cells of angiosarcomas vary from spindle shaped to round or polygonal. Such variation is not unexpected in view of the wide variety *Review of sarcomas of the uterus was made by Drs. S. A. Wolfe, M. Schiffer, and A. Mackles, to whom we are indebted for some of the material included in this report.

1309

PJmOWITZ, PELl\H s. AND GRAYZEL

1310

of ('(•lh; i is often quit(: diffknlL A elassifiration of \·ascular tumors of Htc utel'lls. both benkn and mali!! uant, is ginm in the preceding article. Part L TABLE;

!.

OASES tn• HEMANGlOENDOTHELIO~U UF THE UTERUS REPORTED BINCE

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41 41

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44

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;28

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50

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Died fourth postoperative day Died tenth day; autopsy No follow-up Rapid growth. Untraced Alive 6 J 'ars postoperati \'ely. recurrence in eervical stump, x-ray therapy Died 14 months postopemtively. Autopsy: metasbsis Died in 4 months. Rapid growth of tumor; exploratory laparotomy. Autopsy: metastasis Alive 3 years postoperatively Alive 5% years. Postnll!no· pausal bleeding Alive 5% years. Myomeetomy Alive 5 monthR. Subserous tnmor

~"~"~--'- "·-~~· ~~··

Morphology Hemangioendothelioma.~This neoplasm does not have any g'l'oss characteristic features. The vascular nature of the tumor, however, is apparent from its red-to-purple appearance, although it occasionally may appear to be gray white, due to an absence of red cel1s in the blood vessels. The tumor is generally soft and friable, exhibiting vascular spaces of val·ying size and shape on cut section. In some areas proliferating endothelial cells may be seen to fill completely the lumina of the vessels. The delicate reticular framework of these spaces and the intraluminal growth of neoplastic cells are clearly revealed by silver reticulum stain, which also demonstrates an occult vascularity of the more solid portions of the neoplasm, and characteristic capillary anastomoses not apparent in hematoxylin and eosin preparations are now outlined. Stromal cells consist of elongated and polygonal cells which have grown outside the vascular sheath. These are arranged in cords or solid slieets, a pattern usually reproduced in metastases (Figs. 1-6) . H emangiopericytoma.-While hemangiopericytoma has no distinguishing features, unlike hemangioendothelioma, its usual color is gray white to yellow in spite of its vascular origin. Absence o:f cyanotic-red coloration, so often noted in vascular tumors, is due to the presence of numerous noncanalized

MALIGN ANT VASCULAR TUMORS OF UTERUS

Volume 69 Number 6

1311

proliferating capillaries within the neoplasm, as well as an absence of red blood cells within patent vessels. Occasionally, red cells are present, so that the tumor does have a red coloration. Hemangiopericytomas vary in size from microscopic to 20 em. or more. The smallest tumor of this series was 5 em. and the largest 12 em. Four uterine tumors were submucous, two were intramural but encroaching upon the endometrial cavity, and one was subserous. Absence of a capsule explains the marked difficulty often encountered when enucleation of a tumor is attempted, and hemangioperieytoma is similar, in this respect, to leiomyosarcoma and adenomyoma (Fig. 7).

Fig. 1.

Fig. 2.

47 -491~. Hemanigoencluthelioma. Numerous enrlothelial cells. with attempt a t v es~e l formation. \ Hemat oxylin a nrl eosin . >< 150 ; reduced l;h.) Fig. ~.~Same case. Higher magnification revealing "heaping-up" of endothelial cells in lum en of vessel. (Hematoxylin a nd eosi n. X440; r e
L~S

Diagnosis is based on histologic examination of the tumor. Morphologic variations exhibited by pericytes account for frequently inconstant appearances so charaeteristic of hemangiopericytomas. The cells may be round, ovoid, or spindle shaped, and may contain a round or ovoid vesicular nucleus. Similarity of pericytes to smooth-muscle cells necessitates special differential stains, such as Masson's, to distinguish one type from the other. The cells of a hemangiopericytoma are concentrically arranged around the capillaries of the tumor (Fig. 8). Almost always proliferating capillaries may be noted on careful examination, although in some areas of the tumor these vessels may not be apparent when stained with ordinary hematoxylin and eosin. Silver staining demonstrates noncanalized capillaries, as well as those not apparent because of compression by an overabundance of pericytes. The vessel sheaths are stained black, so that the extraluminal position of the pericytes is emphasized (Figs. 912). In addition, connective-tissue septa may be noted enveloping both large and small aggregates o£ cells and, occasionally, isolated pericytes. The vascular sheaths often vary markedly in thickness, and the reticulum content of the neoplasm also shows considerable variability.

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Volume 69 Number 6

MALIGNANT VASCULAR TUMORS OF UTERUS

A.

1313

B.

Fig. 4.-.4., Same case. Myometrium invade<.l by end oth elia l cells. B, Same case. Silver stain revea ling mar keel vascularity.

A. Fig. 5.-A, S 48·429. Very cellular hemanigoendothelioma. X150; reduced 1,6.) B, Sa.me case. Silver stain to reveal marked vascularity.

B.

(Hematoxylin and eosin.

PEDOWIT7-, E'.l<;LMITH, AND OHAVZf
1314

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l\litotic figures are 1·arely present , but. wheu llt>tt·d i1 ; ln.rge runnlu·rs si!!lll i\ ..apid growth and possible future 111alignant eoursL'. Uccasionallv nests of tumor cells 111av be ohsetved h·ing frc'' within h11 i· phatic channels: lmt their Rignifiran(·t• in' 1h(~ iil>st~IH'P ol'. oh~~ir>u~ ntdns1m~ i~ i" questionable (Fig. J:3l.

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KazJOsi'!$ sarcuma is 1•rimarily a di:;ease of the ski11, more commonly round in males.

No cases ha\·e been rerlorted in whieh the uterus was involved.

Lymphangiosarcoma.-- This tumor, also known as Jymphangioemlotheliollw,

is nonencapsnlated and is composed of endothelial elements with a connectivetissue stroma. Budding endothelial elr.ments give rise to lymphaties of varying size and shape, and the lining cells may proliferate cithet· int.r o- or extraluminally. A highly cellular appearance of some areas of the neoplasm is usuall y the re::mlt of extraluminal proliferatiou. ~~xcept for· oceasional lymphoey1es or clear fluid, the lymph ehannels are empt,v. Lymphangiosarcotlla bears a marked resemblance to hemangioendothelioma, except for absence of red cells within thP ,-essels. Many hernangioendotheliomas, howe\'er, also may he devoid of 1·ed cells. Lymphangiosarcoma most frequently appears in areas where lymph stasis has occurred, such as postmastectomy l:vmphedema,t' and thet·efore would not be expected to he present in the ut~rns: on]~· two rasPs"· 10 involving the ntel'11S ha\'E' been l'qlOrt<'d sillf'C 1!)20 ( 'l'nh]e ffl ) .

Clinical Picture and Treatment Malignant vascular tumors of the uterus most frequently affect wonH.:IL from the middle period of life through the sixth decade, with the greatest inri-

MALIGNANT VASCULAR TUMORS OF UTERUS

Volume 69

Number 6 TABLE

II.

CLINICAL SUMMARY OF

7

CASES 0~' ilEMANGIOPERICY'fOMA OF '£liE UTERUS ~~

THERAPY

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CASES 0~' LYMPHANGIOSARCOMA REPORTED SINCE

Second admission TABLE

+ +

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Recurrence 13 months postoperatively Living 8 years postoperatively. Received x~ray therapy Died 11/2 years postoperatively. Had curettage 2 years and 3 years before vaginal myomectomy; hemangioma was not revealed. Postmenopausal bleeding not controlleu by x-ray therapy + Living t) years poRtoperatively. Had radium inFertion 2 years prior to hysterectomy for ''endometrial polyps.'' RerE>ived postoperative x-ray Living 11 years. Tumor cells in lymphaties. Received postoperative x-ray therapy + Died 1 year postoperatively. Menopause 8 vears before. No metastasis at operation, hut developed pleural metastasis after 7 months. Subserous + Lidng ti months. Tumor cells in lyrnphatirs

+

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Pathologic report, ''fibroid.'' Readmitted 5 years later

+ +

SYMP· TOMS

1920

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dence in the fifth and sixth decades, similar to that of other malignant growths of the corpus uteri. Nulliparous, as well as multiparous, women may have angiosarcomas, and no special significance can be assigned to parity. Metastasis is primarily via the blood stream, although spread of the growth may also occur by direct extension and through the lymphatics. Wides.pread metastasis is common. Diagnosis of angiosarcoma is frequently not made until the removed tumor has been examined histologically. Enlargement of the abdomen may be the first sign noted by the patient. Bleeding, either as hypermenorrhea, polymenorrhea, or postmenopausal, is a common symptom ; its presence is dependent upon the location of the neoplasm in the uterus in relation to the endometrial cavity. Abdominal pain results from degenerative changes or from rapid growth of the neoplasm.

1316

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ft..,ig. 7.- Hcnlangiopf·ricytonul. ( ~ross sectlon of uteru s . N(lh: I H•:";I\ .-,f shnr p tion hetW('f'n tn mr,; · :•nd Hl YO nl f-7'triUtt !.

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Fig. 8.-Hemangiopericytoma. Concentric arrangement of pericytes around capillaries. The "thick-walled" vessel is actually a capillary with condensa ti on of pericytes and connective tissue. (Hematoxylin and eosin. X440; reduced lfo.)

MALIGNANT VASCULAR TUMORS OF UTERUS

Volume 69 Number 6

1317

Fig. 9.

B.

A.

A.

B.

Fig. 10. Fig. 9.-A, S 50-2979. Hemangiopericytoma (malignant). General appearance of tumor; obvious vascularity. (Hematoxyiln and eosin. X 150; r educed l,.f;. ) B, Same tumor stained with Wilder's silver stain. ( X440; reduced l,.f;.) Fig. 10.-A, Same case. Another site from cellular portion ot tumor. Vascularity not apparent. (Hematoxylin and eosin. X150; reduced l,.f;.) B, Wilder's silver stain to reveal previously occult vessel.,. (X440; reduced l,.f;.)

13ifi

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Fig. 11.--.4, liernangiurwricytnrn:t tbt·nt_:,_;J;.J B,

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\Vhenever angiosarcoma is diagno~>ed, the pl'efenc·(l treatment is total hysterectomy with bilateral salpingo-oophorcetomy, e\·ell when metastasis may be present. Since malignancy of hemangiopericytomas oc(•urs in mliy 21 per cent of cases/ 5 myomectomy may be performed, provided there is no evidence of metastasis, in order to preserve fertility. Total hysterectomy and hilattwal salpingo-oophorectomy are indicated, if metastasis has oeenrred. Io he follmve(l by a course of deep x-ray therapy. Such irradiation should also he given post operatively in all cases of hemangioendothelioma, although the valne of post· operative x-ray for these I nrnots has not been conelusiv( ~l.\ demonstrat<·•l. Prognosis Hemangioendothelioma is far more malignant than hemangiopericytoma. Of the 11 cases of hemangioendothelioma reported since 1920, including 3 in the present paper (Table I and II), 4 patients ( 36.4 per cent) died within 14 months after operation; 2 could not be traced (although one may be presumed dead because of the presence of metastasis at operation) ; and 5 remaining patients• were alive from five months to six years after operation. The five-year survival rate in the 11 cases of hemangioendothelioma was 27.3 per cent. Since 1942, when hemangiopericytoma was first described by Stout and Murray,1 6 only 13 cases of this tumor involving the uterus have been noted, including the first 4 cases herein listed.' 2 • 15 • 17 Three additional cases are reported in this paper for the first time, making a total of 16 known cases of hemangiopericytoma of the uterus. Patients 2 and 5 died within one and one-half years after operation from metastasis, and on the basis of local recur-

Voiume 69 Number 6

MALIGNANT VASCULAR TUMORS OF UTERUS

H eman g iopericytomH. Fig. 12.-S 42-474. e xtra luminal position of pericyt es.

Fig. 13.-Same case.

Xormal endometrial linin g of capillaries. (Wilder's s ilver s tain. X440; rt' cluce
Nest of tumor cells lying free within a lymph channel.

1319

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Volume 69 :\lumber 6

MALIGNANT VASCULAR TUMORS OF UTERUS

1321

and metastases. In a case reported by Frank20 a metastatic nodule which occurred 17 years after hysterectomy revealed an identical vascular pattern of the original new growth. Another prominent feature of "stromal myosis" is the presence of thickwalled blood vessels which are thought to be arterioles. Hill, 31 however, has demonstrated that they are actually capillaries with condensation of surrounding connective-tissue fibers and stromal cells. Such vessels are also an outstanding feature of hemangiopericytoma. l\Iany investigators believe that the blood supply of "stromal myosis" is derived from an ingrowth of endometrial vessels of the uterus. Severance of these vessrls should, therefore, produce neerosis in the remaining tumor tissue, unless secondary vascular attachments have been E·stablished. Distant or late recurrence of the tumor following hysterectomy could not obtain its blood supply from the endometrium, and thus would not be expected to reproduce the original cytoarchitecture of the neoplasm, and particular]~, that of the blood vessels. This does occur, however, and suggests an origin of the tumor from vasoformative tissue, such as pericytes, rather than from mature stromal cells. While Embrey 29 and Hill 31 both believe that stromal cells are capable of forming blood vessels, there is no evidence that mature stromal cells can do so. All such neoplasms should be termed hemangiopedcytoma, since similar tumors occur in other organs of the human body as well as the uterus. Hemangiopericytoma is generally benign, and it would be misleading to classify it as endometrial sarcoma rather than as a member of the angiosarcoma group of tumors. To do so would create a falsely grave prognosis for patients with a relatively benign neoplasm.

Summary and Conclusions Review of uterine tumors previously classified as sarcoma and hemangioendothelioma has shown that 10 arc vascular neoplasms: 3 hemangioendotheliomas and 7 hemangiopericytomas. Special stains are essential to delineate the basically vascular structure of angiosarcoma, as well as to distinguish various types of vascular tumors of the uterus. Abnormal vaginal bleeding is the most common symptom associated with angiosarcoma. The preferred treatment for hemangioendothelioma and hemangiopericytoma with metastasis is total hysterectomy and bilateral salpingooophorectomy, followed by deep x-ray therapy. Although hemangioendothelioma is very often malignant, hemangiopericytoma is relatively benign. A review of tumors previously designated ''stromal myosis" revealed their appearance to be identical with hemangiopericytoma, a tumor found in other organs as well as the uterus. For that reason hemangiopericytoma, rather than ''stromal myosis'' or endothelial sarcoma, would better designate the new growth.

References Hemangioendothelioma Surg., Gynec. & Obst. 37: 24, 1923. 2. Singer, S.: Monatsschr. Geburtsh. u. Gynak. 66: 235, 1924. il. Cetroni, M.: Folia gynaec. 27: 567, 1930. 1. Murphy, D. P.:

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4. Valtorta, F.: Clin. ostet. 39: 565, Hli.>i". 5. K.evorkian, A. y·.: New England ;r. l\l\ d. 223: 1, J~J4:0. ti. Stout, A. P.; Ann. ::)urg. 118: 445, 1)'!::. 7. 1\.uoll~ \'\7·. 13.: lTroL & Cutan. Hov. 51: :_~-,, 1047. A:.r. .1. Ollt:T. & (h:-;t·:c. 57: " C'ulwu ...l. fl .. Inglehy, lT., and Uayl. ,I

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An:h. Path. 33: 78, l!t+::. 111. Limbmg, H.: Arch. Gynak. 174: 4ti, 1!14:!.

11 emanyiu pericytorna 11. Pedowitz, P., Felmus, L. B., and Grayzel, D. M.: AM .•T. 0Bt>'r. & GYNJJ:C. 69: 1291, 1955. 1:.!. l'edowit~.• 1'., FelmuH, L. B., and Grayz(•l, (), M.: A~f. .1. OHR'P. & GYNEC. 67: 5-ifl. I!I.).J.. 1:l. Fisht•r, E. R., Kaufnmn, l'\ ., and .Ma~on, K .I.: Am •• 1. Path. 28: HG:l, Hlii:.!. 1+. Hkwart, F., and 'l're.-es, N.: C'aneer 1: ti.+, J!qs, Hi. f\tout, A. l',: Personal communi<'atimJ. Hi. Stout, A. 1'., allll Murray, M. R: "\nn. l-1uq.;. 116: :2!\, lfH-:2. 17. Urt'l'llP, R. R.: Personal <'OllllllUILit~t. & UynaP•'. ~f'etiPn n :2:'5, HlOH. 1!1, l'ltillip~, M. H.: J. Obst. & Gynaee. Hril. Emp. 13: .10::, l!lllt.. ~II. Ji'mnk, R. '1'.: Am. ,J. Caneer 16: J:l:!!i, lfJ:::!. :.!1. llender~ou, ll. X.: AM ..J. 0BST. & UY:a:u. 52: 1000, 19±1). ~~. Uou,Jall, J. R.: .I. Obst. & Gynaoc. B!'it. Ji]mp. 47: n, Hl4il. :2:1. :Mill0r, .J. H., and Tennant R.: J\~1. .J. Ow·n'. & GY~Ec;. 47: 7~4~ l!lJ--4:. 2-1. dnl 'arle, D. W.: \Ve;;t. J. Surg. 53: ·l~, ll.\45. :!5. Park, 'W. W., and 'rennent, R. A.: ,I. OlJRt. & Gynaee. Bril. Emp. 55: ±2~l, 194S. ~li. DaCunha. F.: J. Ohst. & Gynaec. Brit. Emp. 57: 633, 1951l. 27. RolH~rtson, T. 1>., IInntnr, ,V. 0., Lar~nn, C. P., and Sny,ler, G. J\. C.: An1. ,J. ( liu. Path 12: J. J!l~t.l. :2S. Hunter, \V. (' : Surgery 34: 25S, 1!ti3::. 2!1. leloek, ~I. C., and Strand, C. M.: Oh~t. & Gynec. 2: :\84, l95:l. :u. llill, .1. JI.: An·h. Path. 43: 537, lfJH. 1

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