Author
Information
Malviya K*, Fernandes G**, Satia MN***
(*Assistant
Professor,**Associate Professor, Department of Pathology, *** Professor, Department of Obstetrics and Gynecology, Seth GS Medical
College and KEM Hospital ,
Mumbai , India
Abstract
Sertoliform endometrioid carcinoma (SEC) is an unusual ovarian neoplasm that bears histologic similarity to Sertoli and Sertoli-Leydig cell tumors (SLT). We report a rare case of bilateral ovarian sertoliform endometrioid carcinoma in a 60 year old women who presented with postmenopausal bleeding. She underwent total abdominal hysterectomy with bilateral salpingoopherctomy for a right ovarian solid cystic mass. Histopathology revealed features of sertoliform endometrioid carcinoma. On immunohistochemistry, the tumor cells were positive for cytokeratin and negative for calretinin confirming the diagnosis. The differential diagnosis between this tumor and ovarian sex cord- stromal tumor is important since the latter tumors are often benign.
Introduction
Sertoliform
endometrioid carcinoma (SEC) of the ovary is an uncommon variant of
endometrioid carcinoma of the ovary resembling sex cord stromal tumor of pure Sertoli and Sertoli–Leydig cell type.
This entity was first described by Young et al and Roth et al in 1982.[1]
SEC occurs in perimenopausal and postmenopausal women. It is important to
recognize this variant of endometrioid carcinoma and differentiate it from sex
cord stromal tumor because it has good prognosis if confined to the ovary
compared to other endometrioid tumors of the ovary.
Case report
A 60 year old female,
postmenopausal since 10 years, presented to the outpatient department with
complaints of an episode of vaginal bleeding for 10-12 days and soakage of 3-4
pads per day. General and systemic examination were normal. No signs and
symptoms of virilization were seen. On per speculum examination the cervix was
hypertrophied. On vaginal examination, the uterus was anteverted, 6-8 weeks in
size, and there was a right forniceal solid mass 3-4 cm in size adherent to the
posterior uterine wall. Ultrasonography of abdomen showed a right bulky ovary
with bilateral heterogenous echotexture with minimal free fluid. Endometrial
thickness was 10 mm. Computerized tomography scan showed a well defined solid
cystic mass lesion of 6 cm in size arising from the right ovary. Tumor marker
levels (LDH, AFP, CA 125, CEA and Beta HCG) were normal and peritoneal fluid
cytology was negative for malignancy. A total abdominal hysterectomy with
bilateral salpingoophorctomy was performed and the specimen was sent for
histopathological examination.
Gross
Pathology
We received a specimen
of total abdominal hysterectomy with bilateral salpingoopherectomy. The uterus
measured 5x3x2 cm with cervix keratinized. On sectioning the uterus, the
endometrium was 0.2 cm thick and myometrium 2 cm thick. A right ovarian solid
cystic mass with a bosselated surface measuring 6x5x4cm was seen. The capsule
was intact. The cut surface showed a predominantly solid bright yellow neoplasm
with few cystic areas with greenish mucoid material and areas of hemorrhages
were also seen. The left ovary was 2.5x2x1 cm and the cut surface was solid and
yellow in color.
Figure 2. Cut surfaces
of right & left ovary.
Figure 4: Higher
magnification showing anastomosing trabeculae. Individual tumor cells have
vesicular nuclei, prominent nucleoli and moderate cytoplasm (arrow). (H&E x400)
Figure 5: Another
focus showing tubules and tightly packed nests of tumor cells separated by
scanty fibrous stroma (arrow). (H&E x400)
Figure 7 Immunohistochemistry
showing epithelial membrane positivity(arrow). (100x magnification)
Microscopy
Both the ovaries showed
similar histomorphological features of a malignant neoplasm composed of
tubules, anastomosing trabeculae, cords, nests of malignant cells resembling Sertoli
cells set in a fibrous stroma. Individual cells of these cords and tubules
showed vesicular nuclei, prominent nucleoli and mild to moderate cytoplasm.
Areas of conventional endometrioid carcinoma and squamous morules were also
seen focally. The capsule of the tumor was intact.The uterus showed chronic
cervicitis with nabothian cysts and squamous metaplasia and the endometrium
showed cystic atrophy. Immunohistochemistry done, was positive for cytokeratin
and negative for calretinin. Inhibin was not available. Hence the diagnosis of
Sertoliform endometrioid carcinoma was confirmed.
Discussion
SEC were first described
by Young et al.[1] SEC are uncommon variants of endometrioid
adenocarcinoma which bear histologic resemblance to Sertoli and Sertoli Leydig
cell tumor. These are generally seen in postmenopausal patients, in the 60-70
year age group and the patients may have endocrine manifestations. 10% of cases
show bilaterality.[1] 50% of
SLT show endocrine manifestations and patients with SEC can present with virilizing
signs leading to erroneous diagnosis.[2] SEC needs to be
differentiated from SLT since SLT are usually benign. SEC are seen in
perimenopausal and postmenopausal patients while SLT are seen in younger
patients with an average age group of 25 years. Histological features that
differentiates SEC from SLT are presence of areas of conventional endometrioid
adenocarcinoma and presence of mucin at the apical borders of the tumor cells.[3]
SLT stains negative for mucin while SEC stains for mucin. Epithelium of SEC are
pseudostratified while that of SLT are single layered. Other histological
features that favor SEC would be squamoid areas, cilia, presence of
endometriosis and a coexistant adenocarcinoma of endometrium.[4] We
need to differentiate this variant from conventional endometrioid carcinoma
because this variant has better prognosis. Other differential diagnosis for
tumors resembling SEC and SLT include tubular Krukenberg tumor and mucinous
adenocarcinoma, which can be differentiated on histology and special stains.
Immunohistochemistry is
useful when the histology is ambiguous or it may be used for confirmation of
the histological diagnosis. SEC stains for cytokeratin while SLT stains for
inhibin and calretinin.[5] Our case was strongly positive for cytokeratin
and negative for calretinin. Overall, Sertoliform endometrioid carcinoma are
considered low grade malignancies with good prognosis if restricted to the
ovary.
Acknowledgment
We acknowledge Dr.
Pradeep Vaideeswar for his contribution in the diagnosis of this case.
References
- Young RH, Prat J, Scully RE. Ovarian endometrioid carcinomas resembling sex cord-stromal tumors:a clinicopathological analysis of 13 cases. Am J Surg Pathol 1982;6:513-522.
- Jeffrey D, Seidman,
Russel P, Kurman R. Surface epithelial tumors of the ovary. Kurman R. Editor. Blaustein's Pathology of the
Female Genital Tract. 5th ed.
- Misir A, Sur M. Sertoliform endometrioid carcinoma of the ovary. Arch Pathol Lab Med 2007;131:979-981.
- Ordi J, Schammel DP, Rasekh L, Tavassoli FA. Sertoliform endometrioid carcinoma of the ovary: a clinicopathological and immunohistochemical study of 13 cases. Mod Pathol 1999;12:933-940.
- Guerrieri C, Franlund B, Malmstrom H, Boeryd B. Ovarian endometrioid carcinomas stimulating sex cord-stromal tumors: a study using inhibin and cytokeratin 7. Int J Gynecol Pathol 1998;17:266-271
Citation
Malviya K, Fernandes G, Satia MN. Bilateral Ovarian Sertoliform
Endometrioid Carcinoma. JPGO 2015. Volume 2 No. 3. Available from: http://www.jpgo.org/2015/03/bilateral-ovarian-sertoliform.html