A Rare Ovarian Tumor -- Sertoli-Leydig Cell Tumor With Heterologous Element

Rimpy Tandon, MD; Poonam Goel, MD; Pradip Kumar Saha, MD; Navneet Takkar, MD; RPS Punia, MD

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Case Report

A 19-year-old nulliparous woman came to the clinic with complaints of progressive oligomenorrhea of 2 years' duration. She had been amenorrheic for the past 7 months. She had also noticed a change in her voice for 1 year and excessive hair growth on her face, chest, and limbs for the last 2 months. In addition, she complained of vague abdominal discomfort. She was married for the last 4 months and denied any history of anorexia, weight loss, increased libido, or breast recession. Her medical and family history was unremarkable.

Her general physical examination was normal except for the presence of hirsutism and clitoromegaly. Vaginal examination revealed a firm and mobile cystic mass in the left adnexa. An ultrasound examination of the pelvis showed a 7-cm by 7-cm by 5-cm heterogeneous solid cystic mass replacing the left ovary. The right ovary and the uterus were normal. A hormonal profile in blood indicated excessive androgenic activity in the form of elevated serum testosterone level (2 ng/mL; normal, 0.2-1.2 ng/mL); however, levels of dehydroepiandrosterone sulfate (DHEAS), CA 125, and alphafetoprotein (AFP) were normal. Based on these findings, a provisional diagnosis of androgen-producing ovarian tumor was made. The patient underwent ultrasound-guided fine-needle aspiration cytology, which revealed the presence of benign cystic cells. However, because there was a strong suspicion of malignancy, exploratory laparotomy was performed. Operative findings showed replacement of the left ovary by a 7-cm by 6-cm solid, grey-white, smooth-surface mass with intact capsule without spill and with no remnant healthy ovarian tissue. There was no spillage of tumor cells during surgery. The abdominal cavity was explored systematically but there were no deposits anywhere else in the cavity. The para-aortic lymph nodes were not enlarged. The right ovary was found to be slightly enlarged on examination. Left salpingo-oophorectomy was performed, including biopsy from the right ovary. Peritoneal washings were sent for cytologic examination for malignant cells. An omental biopsy was also done during the procedure. However, lymph node dissection was not performed. Frozen section of the resected specimen revealed it to be sex-cord stromal tumor. Gross examination of the pathologic specimen showed that it was an ovarian mass measuring 10 cm by 7 cm by 2 cm. The external surface was smooth. A cut section of the specimen revealed solid as well as cystic areas filled with clear fluid. The histopathologic examination showed a tumor composed of poorly formed cords, nests, and tubules of tumor cells. Tumor cells had hyperchromatic nuclei, a moderate amount of cytoplasm (Sertoli cells), and occasional mitosis. Interspersed between these were nests of polygonal cells with round nuclei and abundant granular cytoplasm (Leydig cells). Focal areas showed dilated glands lined by mucinous epithelium of gastrointestinal type (Figures 1 and 2). Retiform pattern was not seen. Right ovarian biopsy and peritoneal washing did not reveal any abnormal cells.

Figure 1.

Sertoli-Leydig cell tumor. Section shows cords of immature Sertoli cells and clusters of Leydig cells. Haematoxylin & eosin (x 100).

Figure 2.

Sertoli-Leydig cell tumor with heterologous elements. Section shows tumor with heterologous elements showing intestinal-type epithelium. Haematoxylin & eosin (x 200).

Based on the above findings, a final diagnosis of ovarian sex-chord tumor (Sertoli-Leydig cell), stage IA with heterologous elements of gastrointestinal type, intermediate grade, was made. The postoperative period was uneventful and the patient was discharged on the seventh postoperative day.

After the histopathology report was received, we had a detailed discussion of various treatment options with the patient. After her consent, 4 cycles of chemotherapy with a bleomycin, etoposide, and cisplatin regimen were administered 3 times a week. The patient tolerated the chemotherapy well, with no major complications. During 10 months of follow-up, the patient has resumed her periods, with resolution of her virilization symptoms. There is also no increase of her hirsutism. Repeat testosterone levels on follow-up were within normal range.

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