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Journal for Cancer. 115 (2017) 196-202
https://sites.google.com/site/photonfoundationorganization/home/journal-for-cancer
Case Report. ISJN: 5729-5615: Impact Index: 4.17
Journal for Cancer Ph ton
An Unusual Case of Synchronous Carcinosarcoma of the Uterus with
Bilateral Ovarian Papillary Serous Carcinoma and a Leiomyoma
Monal Trisal*
Dharamshila Cancer Hospital and Research centre, New Delhi
Article history:
Received: 14 February, 2017
Accepted: 16 February, 2017
Available online: 09 August, 2017
Keywords:
Carcinosarcoma, Ovarian Papillary Serous Carcinoma,
Leiomyoma
Corresponding Author:
Trisal M.*
Email: monaltrisal ( at ) gmail ( dot ) com
Abstract
Synchronous primary carcinosarcoma of endometrium
and primary serous carcinoma of ovary is an extremely
unique event. We present a very rare case of Uterine
Carcinosarcoma. synchronously presenting with Serous
Carcinoma of the Ovaries in a 58-year-old.
Citation:
Trisal M., 2017. An Unusual Case of Synchronous
Carcinosarcoma of the Uterus with Bilateral Ovarian Papillary
Serous Carcinoma and a Leiomyoma. Journal for Cancer.
Photon 115, 196-202
All Rights Reserved with Photon.
Photon Ignitor: ISJN57295615D859009082017
1. Introduction
Synchronous multiple primary tumors of female
genital tract is a well-known but are rare and have
been reported to compromise 1 to 6 percent of all
genital tract neoplasms (Ayhan A et al;1992). The
most frequent synchronous gynaecological tumors
reported are endometrial and ovarian cancers
(Zaino R et al., 2008). The coexistence of
carcinosarcoma with other gynaecological
malignancies is very rare. We present a very rare
case of Uterine Carcinosarcoma synchronously
presenting with Serous Carcinoma of the Ovaries.
2. Case report
A 58 year old lady came to our hospital with
complaints of post-menopausal bleeding and lower
abdominal pain for 15 days. On imaging by
ultrasonography, abdomen showed mild to
moderate ascites, fibroid uterus and a complex
cystic mass in left ovary. CECT of whole abdomen
revealed bilateral adnexal masses measuring 2.7 x
2.9 cm on the right and 4.5 x 3.3cm on the left side
respectively and enlarged uterus having a
hypodense mass 8.4 x 7.9 x 9.2cm with extensive
omental thickening and ascites. MRI pelvis showed
a large lobulated mass in the endometrial cavity
measuring10.4 x 7.9cm and a cystic mass in left
adnexa (4.5x 3.0cm). Serum biochemistry showed
raised serum CA-125 levels (600U/ml). Ascitic
fluid was examined cytologically was reported as a
metastatic adenocarcinoma. Based on these
findings a clinical diagnosis of ovarian and/or
uterine malignancy was made. Patient was taken up
for cytoreductive surgery. Per-operatively uterus
was bulky, with enlarged bilateral ovaries,
omentum showed caking and deposits were seen on
recto-sigmoid, small bowel, peritoneum and
appendix. Omentum was sent for intraoperative
consultation and reported as metastatic deposits of
Papillary Serous Carcinoma with Psammoma
bodies. On gross examination, the uterus was
distorted with tumor deposits on surface and
measured 17.0 x 16.0 x 8.5 cm. The uterine cavity
showed a shaggy grey brown heterogenous tumor
measuring 14cm in maximum dimension,
distending the uterine cavity with thinned out walls
and reaching upto the internal os of cervix. An
intramural fibroid (4.5 x 5 cm) was also seen.
(Figure 1)Right and left ovarian masses measured
3.0 cm x 2.5cm x 2.5cm and 4.0 x 3.5 x 2.5cm
respectively and showed solid, cystic and papillary
areas.(Figure 2). Omentum (30.0 x 27.0cm)
showed tumor deposits the largest measuring 6.0
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x5.0 cm. Microscopic examination revealed a High
Grade Papillary Serous Carcinoma (Figure
3a,3b,3c) involving bilateral ovaries, right
fallopian tube and bilateral parametria with surface
deposits on uterine serosa extending into
myometrium, along with metastatic deposits in
omentum and rectosigmoid. The uterine cavity
tumor had a biphasic tumor morphology with
pleomorphic malignant epithelial and mesenchymal
components which revealed rhabdomyoblastic
differentiation. This was reported as a
Carcinosarcoma (Malignant Mixed Mullerian
Tumor). (Figure 4a,b,c) The metastatic deposits in
omentum, small bowel biopsy, urinary bladder
biopsy and appendix were also similar and biphasic
in nature. Thus, omentum had metastasis from both
ovarian and uterine tumors. Lymphvascular
invasion was also present in the high grade,
papillary serous carcinoma. (Figure 3d). One out of
eight left pelvic lymph nodes (1/8) and three out of
thirteen para-aortic lymph nodes (3/13) were
involved by Serous Carcinoma with extranodal
extension. Eight right pelvic lymph nodes were
uninvolved. Pathological stage for Serous
Carcinoma was pT3cN1. On Immunohistochemistry,
the ovarian tumor cells expressed WT1 and ER. In
the uterine tumor the epithelial cells expressed CK
whereas stromal cells showed focal positivity for
Vimentin with some areas expressing SMA and
Desmin.(Figure 5) No expression of ER, GFAP or
S-100 was seen in the spindle cells.
The final report was High Grade Papillary Serous
carcinoma of bilateral ovaries with Carcinosarcoma
of Uterus and Leiomyoma of the Uterus.
3. Discussion
Synchronous endometrial and ovarian primary
cancers are extremely rare and the reported
incidence rates ranges from 2 to 8.5%.
Synchronous tumors of the endometrium and ovary
can be classified into 3 groups namely, endometrial
cancer with metastasis to ovary, two independent
primary tumors & ovarian cancer with metastasis to
the endometrium, which is the least
common(Pamela T. Soliman et al., 2004). The
etiology of synchronous tumors remains unclear
but the theory of ‘secondary mullerian system’
suggests that the epithelia of cervix, uterus,
fallopian tubes, ovaries and peritoneal surfaces
share embryologically similar mullerian tissues and
therefore might develop synchoronous tumors
when exposed to some carcinogens (Eifel P et al.,
1982). Some authors suggest that neoplasms
originating in dysplasia share hormonal receptors
and may cause multiple primary tumors in
histologically similar tissues.1 It has been clearly
demonstrated that some of the synchronous
neoplasms in female genital tract are related to
familial cancer syndromes or genetic mutations. In
the female genital tract, synchronous tumors make
0.63% of all genital malignancies (Momcilo D et
al., 2007).
The most frequent synchronous gynaecological
tumors reported are synchronous endometrial and
ovarian cancers. Carcinoma of endometrium can
occur simultaneously in about 10 percent of
patients with ovarian carcinoma (Zaino R et al.,
2001). Rare cases of uterine carcinosarcomas and
leiomyosarcomas associated with ovarian
carcinomas have been reported in literature (Iris M.
Castro et al; 2000). The identification of
synchronous ovarian malignancies in women with
endometrial carcinoma is complicated by the fact
that the ovary represents a possible site of
metastatic spread from the endometrium.
Consequently, it is particularly difficult in women
with tumors of identical histology. These women
comprise only a minority of the patients and
possibly may simply have ovarian metastasis from
the endometrium. Carcinosarcomas account for
almost half of uterine sarcomas and most cases are
postmenopausal as in our case. The most common
presenting symptoms are abnormal uterine
bleeding, abdominal pain and abdominal fullness.
Carcinosarcomas are described as typically large,
bulky polypoidal masses filling the uterine cavity
and prolapsing through the cervical os (D’ Angelo
E; 2010). Microscopically, Carcinosarcomas are
composed of both epithelial and mesenchymal
components may be intimately mixed or may be
seen as two distinct components. The epithelial
component mainly composed of serous (two-thirds
of cases) or endometrioid carcinomas (one-third of
the cases). Sarcomatous components may be
homologous, containing cells native to the uterus
including stromal sarcoma, fibrosarcoma or
leiomyosarcoma (2%) or heterologous with mixed
components including rhabdomyosarcoma(18%),
chondrosarcoma (10%), osteosarcoma (5%) or
liposarcoma (1%) (Ferguson SE; 2007). In the
present case whether the ovarian tumors were
metastatic epithelial component or synchronous
second primary needed to be established. Besides
the dissimilar histological findings of two tumor
types, the ovarian masses were grossly different
from uterine tumor and were partly cystic with
papillary areas. Microscopically the endometrial
carcinosarcoma showed a biphasic pattern with
epithelial component admixed with heterologous
mesenchymal stromal component showing
rhabdomyoblastic differentiation, with absence of
branching papillae and psammoma bodies. Ovarian
tumor showed a classical picture of papillary serous
carcinoma of the ovary with branching papillae and
psammoma bodies. On the other hand, no
sarcomatous component was found in the ovarian
tumor despite a thorough
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Figure 1: Gross appearance of distorted uterus with tumor deposits on surface and uterine cavity showing a shaggy grey
brown heterogenous tumor and an intramural fibroid.
Figure 2: Gross appearance of TAH-BSO specimen showing left ovarian mass showing solid, cystic and papillary areas and
surface deposits.
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Figure 3: Microscopic examination of ovaries showing complex branched papillae (a,b), psammoma bodies (c) and
lymphovascular invasion (d) (40x, H&E) consistent with a High Grade Papillary Serous Carcinoma
(a) (b)
(c) (d)
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Figure 4: Microscopic examination of uterus showing a biphasic tumor morphology with pleomorphic malignant epithelial
and mesenchymal components (a,b) (10x, H&E; 40x, H&E respectively) with rhabdomyoblastic differentiation (c) (40x,
H&E) consistent with Carcinosarcoma.
(a) (b)
(c)
Figure 5: On Immunohistochemistry, in the uterine tumor epithelial cells expresses CK whereas stromal cells shows
positivity for Vimentin, SMA and Desmin in large cells (rhabdomyofibroblastic differentiation) and the ovarian tumor cells
express WT1 and ER.
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examination. Psammoma bodies were prominent
feature of the primary tumors and the metastatic
sites like uterine surface. On
immunohistochemistry, the epithelial component of
Carcinosarcoma expressed CK whereas stromal
cells expressed Vimentin with focal expression of
SMA and Desmin. No expression of ER was seen.
On other hand the epithelial cells of Serous
Carcinoma of the ovaries expressed ER with
nuclear WT1 positivity. Among the synchronous
uterine and ovarian neoplasms, the behaviour of
MMMTs is significantly worse than FIGO grade 3
serous carcinomas, endometrial carcinomas and
clear cell carcinomas(A. E. Bland; 2007). Cases in
which the carcinomatous component was serous or
clear cell carcinoma had a significantly greater
tendency to be associated with an increased
frequency of metastatic disease. The histologic
appearance of the sarcomatous component
(homologous versus heterologous element, grade of
sarcoma) of MMMTs does not appear to have any
prognostic significance (S.E. Ferguson; 2007).
Hence, there is an absolute need to distinguish
between the two groups for the purpose of
prognostication and to decide whether the two
tumors are independent primaries, or whether one
is a primary and the other a secondary, in which
case, which one of them is the primary.
Conclusion
Summarising, synchronous primary
carcinosarcoma of endometrium and primary
serous carcinoma of ovary is an extremely unique
event. Simultaneous detection of malignancy in
endometrium and ovary often challenges the
clinician and pathologists to recognize these
combinations of tumors to avoid their
misinterpretation as a combination of primary and
metastatic tumors and plan appropriate
managements and evaluate prognostic implications.
Research Highlights
Synchronous Primary Carcinosarcoma of
endometrium and primary serous carcinoma of
ovary is very rare. Often challenges the clinician
and pathologists to recognize these combinations of
tumors to avoid their misinterpretation as a
combination of primary and metastatic tumors and
plan appropriate managements and evaluate
prognostic implications.
Competing Interests
The authors declare that they have no conflicts of
interest.
Authors’ Contributions
All authors contributed equally, read and approved
the final manuscript.
References
A. E. Bland., R. Stone., C. Heuser et al., 2009. A clinical
and biological comparison between malignant mixed
mullerian tumors and grade 3 endometrioid endometrial
carcinomas. International Journal of Gynecological
Cancer, 19(2):261-65.
Ayhan A., Yalcin O.T., Tuncer Z.S., Gurkan T.,
Kucukali T., 1992. Synchronous primary malignancies
of the female genital tract. European Journal of
Obstetrics andGynaecology and Reproductive Biology,
45:63-6.
D’ Angelo E., Prat J., 2010. Uterine Sarcomas- A review.
Gynecology and Oncology Journal, 116:131-139.
Eifel P, Hendrickson M., Ross J., Balon S., Martinez A.,
Kempson R., 1982. Simultaneous presentation of
carcinoma involving ovary and the uterine corpus.
Cancer, 50, 163-70.
Ferguson SE., Tornos C., Hummer A., Barakat RR.,
Soslow RA., 2007. Prognostic features of surgical stage I
uterine carcinosarcoma. American Journal of Surgical
Pathology, 31(11), 1653-61.
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Iris M., Castro., Philip P.Connel., Steven Waggoner.,
Jacob Rotmensch and Arno J. Mundt., 2000.
Synchronous Ovarian and Endometrial Malignancies.
Journal of Clinical Oncology, 521- 525.
Momcilo D., Slobodanka M., Gordana D., Bozidar J.,
2007. Endometrioid tumor of the ovary and uterus,
metastasis or not-Case Report.ActaMedicaMediterranae,
47(4), 15-19.
Pamela T., Soliman., Brian M., Slomovitz., Russel R.,
Broadders., Charlotte C Sun., Jonathan C Oh., Patricia J.
Elifel., David M. Gershenson and Karan H. hu., 2004.
Synchronous primary cancers of the endometrium and
ovary: a single institution review of 84 cases.
Gynecology Oncology, 94, 456-462.
S. E. Ferguson., C. Tornos., A. Hummer., R. R. Barakat.,
and R. A. Soslow.,2007. Prognostic features of surgical
stage I uterine carcinosarcoma. American Journal of
Surgical Pathology, 31(11), 1653–61.
Zaino R., Whitney C., Brady M.F., De Geest K., Burger
RA., Buller RE., 2001. Simultaneously detected
endometrial and ovarian carcinomas-a prospective
clinicopathologic study of 74 cases: a gynecologic
oncology group study.,Gynecologic Oncology, 83:355–
62.
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