Endocrine Abstracts

Introduction: Steroid cell tumors are rare ovarian sex cord-stromal tumors with malignant potential comprising <0.1% of all ovarian tumors. A subtype called not otherwise specified (NOS) accounts for approximately one-half of all ovarian steroid cell tumors. The average age of presentation is 43 years, with androgenic clinical features in 56–77% of cases.

Case report: A 21-year-old female, on liver transplantation waiting list due to Niemann–Pick disease, was referred to an endocrinology appointment in November 2013 for hirsutism and acne with 6 months of evolution and progressive worsening. Male pattern baldness, acne, severe hirsutism and clitoromegaly were noticed on physical examination. Laboratory workup revealed: total testosterone 15.89 ng/ml (0.04–0.8), free testosterone 34.74 pg/ml (<2.57), androstenedione >10 ng/ml (0.3–3.3), 17-hydroxyprogesterone 3.5 ng/ml (0.2–1.8), and DHEA-S 16 μg/dl (35–4300. During ultrasound study in August 2013 a right adnexal mass was noted leading to an MRI, which revealed a large 6.4×5.2×5 cm ovarian mass, with normal adrenal glands. Tumor markers were negative. The patient underwent surgery: a small amount of ascitic fluid was evacuated and right salpingo-oophorectomy was performed. No other alterations on the abdomino-pelvic cavity were found. Histopathological examination revealed an ovarian steroid cell tumor NOS, with some features increasing the risk of malignancy (tumor diameter >7 cm, mitotic activity, hemorrhage). Peritoneal fluid was negative for malignancy. Three weeks after surgery androgen level became normal and acne improved.

Conclusion: Androgen-producing tumors should be suspected in women with virilization and high testosterone levels. Approximately one third of steroid cell tumors NOS are malignant, 6% are bilateral and little is known about their behaviour. This case is significant because of the rarity of these tumors in a young age. In this patient a close surveillance is required due to the perspective of hepatic transplantation and immunosupressive therapy in the short term.