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Müllerian adenosarcoma of the uterus with pure angiosarcoma: Case report
Case Studies MiiLLERlAN
ADENOSARCOMA
OF THE UTERUS
WITH
PURE
ANGIOSARCOMA:
CASE
REPORT
E:KNUI. E. 12x~. MD, PIN(:A~ BII-IIKMAN, MI), ANI)JAMEST. SIINI)EE:N.MI)
M.1,re$ort a (‘USC o/ miilie?‘ian adenosarcoma nfthe utrrx~ with benign endometnoid epithelial romponmt und histologically pure ungio.m rroma. Thi, seemingly unique .surcom.atous ingredient, among uterine and c~strautrrine adeno.tarroma~ reported in the English literature. i.c conszd~~rd to hr u homologous component of the tumor. Thr patirnt, (1 7 I-yrctr-old woma?z with a several-month history qf intermittent :uaginal bleeding, was found at abdominal hysterectomy to hazle a large polypid intrauterkw tumor with super-&l extension into the mymetrium. The patient was treated with h,(100 rud of ndjurvznt radiation hut died n J>w months after of what rlinicully appewed to 1)~massive abdominal bleedi,ng. The literature regurding uterine ttrrn~~~rc classified u.7 angiosarcoma or malignclnt hemangroendothrllr)mcl iv hrirfly reviewed. HCJM P..i7HoI. 32: 12891_7‘JI. (:opvr-ight C I99i by W.H. Saunders C:ornpnn~
Malignant mixed miillerian tumors (MMMTs) of the endomc~rium art’ hiphasic neoplasms composed of a malignant epithelial con1ponrnt, usually grade II or III endometrioid adeiioc.ai-cilioma.’ and a malignant stromal component that may he homologous or heterologous. The homologous component is often ;I high-
01:
-1 t’:ASIC
A prc~:it~slv hcalthp, gravida 2, para 1, 7 1-year-old woman presented wit11 profuse vaginal bleeding of 2 weeks’ duration; ;I history revealed intermittent vaginal Herding foi- at least 8 ~no~lths. A dilatation and curettage had been performed 6 months lmot- to admission. but was nondiagnostic. Past medical llistorv revc&d mild hvpertension that was controlled with
1289
diuretics. On physical examination, the patient had intravaginal blood clots and an irregular enlarged uterus. 1.ahoratot-y srudies showed iron deficiency anemia and hypoalbuminemia. A repeat endometrial curettage yielded a biphasic malignant neoplasm, and an abdominal hysterectomy with salpingo-oophorectomy was performed. Bone, liver, and spleen scans were normal. The patient subsequently underwent several courses of radiation therapy (total, 6,000 rad). Two months following hospital discharge, the patient was found at home unresponsive; she died shortly thereafter at the hospital. Clinically, the terminal event was felt to be secondary to massive abdominal bleeding, but an autopsy was not performed Gross Pathology l’he second endometrial curetting measured gregate and the histologic findings were identical in rhe hysterectomy specimen. The endometrial greatly distended by a 7-cl11 polypoid tumor that necrotic and involved only the superficial one’ mvonie~~-ium. .Also present were multiple utel-inc measuring up to 2 cm in diametrl-. Microscopic
Patholog
4 cm in agto those seen cavity was was part+ third of tt1t leioni~onia~
and ImmunohistoloLgy
The tumor was an MA, with the epith.elial component c-onsisting of benign endometrioid glands spaced relativel) evenly throughout a malignant stromal component, which was a pure angiosarcoma (Fig 1A and B). The stromal sarcoma was composed of small, irregular vasoformative channels lined by hyperchromatic endothelial cells that in some areas formed distinctive micropapillary tufts (Fig 1C); in other foci, the vasoformative component had a more solid arrangement. Areas of’ necrosis and hemorrhage were present superficially within the tumor. The vascular nature of the sarcomatous stroma was confirmed by diITuseIy positive immunostaining for factor VIIIrelated antigen (Fig 1D; Dako Corporatiorl. Sant;l Barbara, (:A; dilution. 1 :2,0(H)).
Miillerian adenosarcomas are neoplasms with Iow malignant potential in contrast to MMMTs. In a recent review of 100 cases of MA of the uterus, recurrent ttmlor composed of pure sarcoma or adenosarcoma was documented in 26% of patients and was usually confined to the vagina, pelvis, or abdomen; blood-borne distant metastases wcrc evident in onl) two cases.” These tmnors can also he seen in extrauterine site;, such as the ovary. ancl elsewhere in thr pelvis,“’ ” but approximately 87% of cases of MA arise in the endometrium, !)% in the enclocervis, and 4Yo in the myometrium.” In a ret-ent survey of 220 MAs reported in the English literature. the tumors werr typically biphasic, with the glandular component being clearly henigL or only milclly atypical.‘~“.” In most cases, the stromal component is a low-grade et~dometrial strolnal sarcoma or fibrosarconia. but occasionally ;I 5tronial c~,niponent showing smooth muscle tlifferentiatic~n niay be present. ‘The example of an MA reported here appears to be ulliclue iii that the sti~omal comporien~ was a prire angiosal-c oma. Bt--
FIGURE 1. (A) An MA of the uterus is composed of benign-appearing endometrial glands set within a mesenchymal stroma that is purely angiosarcoma. (Hematoxylin-eosin stain; magnification X 108.) (B) A closer view of the angiosarcoma shows tightly packed vascular channels lined by plump, atypical endothelial cells. In some areas, vasoformative tendency was difficult to discern because of a more solid arrangement of cells. (Hematoxylin-eosin stain; magnification x360.) (C) An area of MA showing papillary projections lined by malignant endothelial cells, (Hematoxylin-eosin stain: magnification x400.) (D) Positive immunostaining for factor VIIIrelated antigen accentuates the endothelial component. (Streptavidin alkaline phosphatase method; magnification X400.)
CASE STUDIES nign rnclomc~1rioid glandular elenrcnts wet-e diffusely scattered Ih~oughout ilie nec@asni, even in sites of super-ficial niyoInc~trial invasion. In analyzing all cases of MA reporlrd in the I-ecenl littmturr review by Clement and Srully, as wrll as a ft~ casc5 reportrd subscqurn~ly. there is no mention of a ~otnpotien~ of a~lgiosarcoma or malignant tirniar~gioendottrc~lioma in any 01 the tumors”: sitnilarly. ‘‘I rcvirw f )f several of the larget~ t-eportcd series of MMMTs makes no I-cferencr I0 angiosart oma, Cithcr as a homologous or heterologous ingrc*dicnr.‘,“’ ” I( lias been I-eported that liererologous nicscnchvmal clcmellts are encountered in approsiniately one cluartcr 01 (‘;ISCS01‘ RIA, with thv most common being I-hat,do~i~~c~sa~-~c~~t~ia; lixi ,)L fCta-typr cartilagr. adipose hue. li,xKll’c,nlla. mti wen a component suggesting neurcqithelial ditfrrentiati~rm tiavr also brcn noted.” Sarc‘omatous over~q-owlh 01 MA has been rrpa)rted in ;I minority of cases. which ma\ c’ontl.ibute to clinical aggr-essiveness with tunior rc’cIii-rence OI mt,tasUsis.‘” The pro”iinrnl angiosai-coma component iii our case of MI is rrgardcd as ;I homologous ingredient of the tumot- given the constant pue~~c e 01 vascular structures in the endomert.tu111, IOWCI urcrirtr segment. and myornetriuru. as well as ,)I tlrr sites \+here M.\s have been described. Angiosarcomas 01 malignan ti~~~ran~ioendotheliornas are rare in the female gcmital ~rac’t. th~sr tumors have heen documen~cd in the uterus.’ ” but not as a component of MA, as in our case. An 0~ casional (3ample $)I‘uterine helnangioperi~vtorria has also A rccc’n1 rt4ew of the wc)rl;i literature rebc.elt q~c~rrcd” vc,alrtl IiinC c ascs of ;mgic,sat-coma or malignant hetliangioend~~tlwlio~~~~ of 111~utcxrus. wilt1 vaginal hlreding being the mos( c~~mtnonpl,csenting Illanifestation. In approxinlatvly half ot tl~ GISCS.tttrre was local recurrencr or death due to mecastatic lum),~. t),rc Iollow-uf, it1 several paticnrs was incomple~r.” Thr p.cticnt ill stir I-t:port with uterine MA diet1 sudtlenh and ml~sp~ctt~ll~ 2 nronths following tlischarge ft-om the. hospital. atId was c.litlicallv suspectrd 10 have massive iiitr;l-,il~domirlal I)lecditig; tiowC\c~I-. an autopsv was not pcrfornied. Ic2ving ,I,,attswered vrherkr the patient had a local rccurrcncv or ahdc~niinal bpwxl t,y tumor. 7Iw shortest interval to rrcurwril hIA in ~hr rcsvitw tn Clement and Scully was 6 n~onths (awra~e. 3 4 \(.;It.\l.‘4
ADENOSARCOMA
OF THE UTERUS
WITH
PURE
ANGIOSARCOMA:
CASE
REPORT
E:KNUI. E. 12x~. MD, PIN(:A~ BII-IIKMAN, MI), ANI)JAMEST. SIINI)EE:N.MI)
M.1,re$ort a (‘USC o/ miilie?‘ian adenosarcoma nfthe utrrx~ with benign endometnoid epithelial romponmt und histologically pure ungio.m rroma. Thi, seemingly unique .surcom.atous ingredient, among uterine and c~strautrrine adeno.tarroma~ reported in the English literature. i.c conszd~~rd to hr u homologous component of the tumor. Thr patirnt, (1 7 I-yrctr-old woma?z with a several-month history qf intermittent :uaginal bleeding, was found at abdominal hysterectomy to hazle a large polypid intrauterkw tumor with super-&l extension into the mymetrium. The patient was treated with h,(100 rud of ndjurvznt radiation hut died n J>w months after of what rlinicully appewed to 1)~massive abdominal bleedi,ng. The literature regurding uterine ttrrn~~~rc classified u.7 angiosarcoma or malignclnt hemangroendothrllr)mcl iv hrirfly reviewed. HCJM P..i7HoI. 32: 12891_7‘JI. (:opvr-ight C I99i by W.H. Saunders C:ornpnn~
Malignant mixed miillerian tumors (MMMTs) of the endomc~rium art’ hiphasic neoplasms composed of a malignant epithelial con1ponrnt, usually grade II or III endometrioid adeiioc.ai-cilioma.’ and a malignant stromal component that may he homologous or heterologous. The homologous component is often ;I high-
01:
-1 t’:ASIC
A prc~:it~slv hcalthp, gravida 2, para 1, 7 1-year-old woman presented wit11 profuse vaginal bleeding of 2 weeks’ duration; ;I history revealed intermittent vaginal Herding foi- at least 8 ~no~lths. A dilatation and curettage had been performed 6 months lmot- to admission. but was nondiagnostic. Past medical llistorv revc&d mild hvpertension that was controlled with
1289
diuretics. On physical examination, the patient had intravaginal blood clots and an irregular enlarged uterus. 1.ahoratot-y srudies showed iron deficiency anemia and hypoalbuminemia. A repeat endometrial curettage yielded a biphasic malignant neoplasm, and an abdominal hysterectomy with salpingo-oophorectomy was performed. Bone, liver, and spleen scans were normal. The patient subsequently underwent several courses of radiation therapy (total, 6,000 rad). Two months following hospital discharge, the patient was found at home unresponsive; she died shortly thereafter at the hospital. Clinically, the terminal event was felt to be secondary to massive abdominal bleeding, but an autopsy was not performed Gross Pathology l’he second endometrial curetting measured gregate and the histologic findings were identical in rhe hysterectomy specimen. The endometrial greatly distended by a 7-cl11 polypoid tumor that necrotic and involved only the superficial one’ mvonie~~-ium. .Also present were multiple utel-inc measuring up to 2 cm in diametrl-. Microscopic
Patholog
4 cm in agto those seen cavity was was part+ third of tt1t leioni~onia~
and ImmunohistoloLgy
The tumor was an MA, with the epith.elial component c-onsisting of benign endometrioid glands spaced relativel) evenly throughout a malignant stromal component, which was a pure angiosarcoma (Fig 1A and B). The stromal sarcoma was composed of small, irregular vasoformative channels lined by hyperchromatic endothelial cells that in some areas formed distinctive micropapillary tufts (Fig 1C); in other foci, the vasoformative component had a more solid arrangement. Areas of’ necrosis and hemorrhage were present superficially within the tumor. The vascular nature of the sarcomatous stroma was confirmed by diITuseIy positive immunostaining for factor VIIIrelated antigen (Fig 1D; Dako Corporatiorl. Sant;l Barbara, (:A; dilution. 1 :2,0(H)).
Miillerian adenosarcomas are neoplasms with Iow malignant potential in contrast to MMMTs. In a recent review of 100 cases of MA of the uterus, recurrent ttmlor composed of pure sarcoma or adenosarcoma was documented in 26% of patients and was usually confined to the vagina, pelvis, or abdomen; blood-borne distant metastases wcrc evident in onl) two cases.” These tmnors can also he seen in extrauterine site;, such as the ovary. ancl elsewhere in thr pelvis,“’ ” but approximately 87% of cases of MA arise in the endometrium, !)% in the enclocervis, and 4Yo in the myometrium.” In a ret-ent survey of 220 MAs reported in the English literature. the tumors werr typically biphasic, with the glandular component being clearly henigL or only milclly atypical.‘~“.” In most cases, the stromal component is a low-grade et~dometrial strolnal sarcoma or fibrosarconia. but occasionally ;I 5tronial c~,niponent showing smooth muscle tlifferentiatic~n niay be present. ‘The example of an MA reported here appears to be ulliclue iii that the sti~omal comporien~ was a prire angiosal-c oma. Bt--
FIGURE 1. (A) An MA of the uterus is composed of benign-appearing endometrial glands set within a mesenchymal stroma that is purely angiosarcoma. (Hematoxylin-eosin stain; magnification X 108.) (B) A closer view of the angiosarcoma shows tightly packed vascular channels lined by plump, atypical endothelial cells. In some areas, vasoformative tendency was difficult to discern because of a more solid arrangement of cells. (Hematoxylin-eosin stain; magnification x360.) (C) An area of MA showing papillary projections lined by malignant endothelial cells, (Hematoxylin-eosin stain: magnification x400.) (D) Positive immunostaining for factor VIIIrelated antigen accentuates the endothelial component. (Streptavidin alkaline phosphatase method; magnification X400.)
CASE STUDIES nign rnclomc~1rioid glandular elenrcnts wet-e diffusely scattered Ih~oughout ilie nec@asni, even in sites of super-ficial niyoInc~trial invasion. In analyzing all cases of MA reporlrd in the I-ecenl littmturr review by Clement and Srully, as wrll as a ft~ casc5 reportrd subscqurn~ly. there is no mention of a ~otnpotien~ of a~lgiosarcoma or malignant tirniar~gioendottrc~lioma in any 01 the tumors”: sitnilarly. ‘‘I rcvirw f )f several of the larget~ t-eportcd series of MMMTs makes no I-cferencr I0 angiosart oma, Cithcr as a homologous or heterologous ingrc*dicnr.‘,“’ ” I( lias been I-eported that liererologous nicscnchvmal clcmellts are encountered in approsiniately one cluartcr 01 (‘;ISCS01‘ RIA, with thv most common being I-hat,do~i~~c~sa~-~c~~t~ia; lixi ,)L fCta-typr cartilagr. adipose hue. li,xKll’c,nlla. mti wen a component suggesting neurcqithelial ditfrrentiati~rm tiavr also brcn noted.” Sarc‘omatous over~q-owlh 01 MA has been rrpa)rted in ;I minority of cases. which ma\ c’ontl.ibute to clinical aggr-essiveness with tunior rc’cIii-rence OI mt,tasUsis.‘” The pro”iinrnl angiosai-coma component iii our case of MI is rrgardcd as ;I homologous ingredient of the tumot- given the constant pue~~c e 01 vascular structures in the endomert.tu111, IOWCI urcrirtr segment. and myornetriuru. as well as ,)I tlrr sites \+here M.\s have been described. Angiosarcomas 01 malignan ti~~~ran~ioendotheliornas are rare in the female gcmital ~rac’t. th~sr tumors have heen documen~cd in the uterus.’ ” but not as a component of MA, as in our case. An 0~ casional (3ample $)I‘uterine helnangioperi~vtorria has also A rccc’n1 rt4ew of the wc)rl;i literature rebc.elt q~c~rrcd” vc,alrtl IiinC c ascs of ;mgic,sat-coma or malignant hetliangioend~~tlwlio~~~~ of 111~utcxrus. wilt1 vaginal hlreding being the mos( c~~mtnonpl,csenting Illanifestation. In approxinlatvly half ot tl~ GISCS.tttrre was local recurrencr or death due to mecastatic lum),~. t),rc Iollow-uf, it1 several paticnrs was incomple~r.” Thr p.cticnt ill stir I-t:port with uterine MA diet1 sudtlenh and ml~sp~ctt~ll~ 2 nronths following tlischarge ft-om the. hospital. atId was c.litlicallv suspectrd 10 have massive iiitr;l-,il~domirlal I)lecditig; tiowC\c~I-. an autopsv was not pcrfornied. Ic2ving ,I,,attswered vrherkr the patient had a local rccurrcncv or ahdc~niinal bpwxl t,y tumor. 7Iw shortest interval to rrcurwril hIA in ~hr rcsvitw tn Clement and Scully was 6 n~onths (awra~e. 3 4 \(.;It.\l.‘4