Sertoliform Endometrioid Carcinomas of the Right Ovary

Abstract

Abstract Background: Endometrioid carcinomas of the ovary are a sub-type of epithelial ovarian tumors. The vast majority are malignant and invasive. On imaging, they are usually characterized as complex nonspecific solid-cystic masses and found associated with endometriosis. However, endometrioid carcinoma of ovary resembling sex cord-stromal tumor is a rare variant of endometrioid adenocarcinoma that focally looks like a sex cord-stromal tumor with sertoli, leydig, or granulosa cells. Sertoliform endometrioid carcinoma of the ovary (SEC) is one variant that bears histologic similarity to Sertoli and Sertoli-Leydig cell tumors (SLTs). Clinical Case: A 47 year old, premenopausal female, presented with rapid growing hirsutism, frontal hair loss, abdominal distension and constipation for less than one year. On clinical examination modified Ferriman-Gallwey (mFG) score was 17/36. Abdominal examination showed a palpable mass in lower abdomen, 25cm in size, rounded, and hard in nature. CT abdomen and pelvis revealed large pelvic abdominal mass (21x20.5x17 cm) with cystic and basal soft tissue components related to right ovary. Preoperatively laboratory investigations showed FSH 9.96 mIU/mL, LH 15.4, Estradiol (E2) 94.7 pg/ml, raised total testosterone 4.38 nmol/l, normal SHBG 34 nmol/l and normal DHEA-S 161 microg/dl. Total abdominal hysterectomy and bilateral salpingo-oophorectomy was done, in which the right ovary was seen to be replaced by a solid cystic tumor with ruptured capsule. Grossly, the right ovary was enlarged measuring 9.5 cm × 8 cm × 4.8 cm. Microscopy showed round to solid tubules lined by pseudostratified columnar epithelium with elongated nuclei resembling sertoli tumor-like pattern along with conventional endometrioid tumor. A histopathological diagnosis of SEC of right ovary was made. Immunohistochemistry showed tumor cells strongly immunoreactive for epithelial membrane antigen (EMA), cytokeratin (CK) but negative for inhibin, thus confirming the diagnosis of SEC of right ovary. Conclusion: SEC is extremely rare variant of endometrioid carcinomas and tend to present at an earlier stage as compared with most endometrioid carcinomas of the ovary. Recognition of SEC in virilizing patients is important as it is a well-differentiated, low-grade malignancy that displays a good prognosis when confined to the ovary.