(93) Ovarian Hyperthecosis Resulting in Torsion in a Virilized Adolescent Female: A Case Report

Background: Ovarian hyperthecosis (OH) results in severe hyperandrogenism or virilization, elevated levels of circulating androgens, and bilaterally enlarged ovaries. This clinical picture largely overlaps with polycystic ovarian syndrome (PCOS), the most commonly diagnosed condition in adolescent females who present with signs of androgen excess and oligomenorrhea. OH is primarily found in the postmenopausal population and is rare in adolescents; thus virilizing symptoms in an adolescent with a pelvic mass prompts concern for an androgen-secreting tumor. Here, we report an unusual presentation of a 15-year-old female with virilization who presented with a large pelvic mass and was found to have ovarian hyperthecosis resulting in adnexal torsion.

Case: A 15-year-old Caucasian female with a history of primary amenorrhea in the setting of obesity, hyperinsulinemia, and virilization with deepened voice and clitoromegaly for the past year was referred to Gynecology after imaging revealed large complex bilateral ovarian cysts. MRI showed a 9x7x9 cm midline mass arising from the right ovary with internal septations and small mural enhancing nodules concerning for malignancy. Laboratory evaluation revealed a marked elevation in total testosterone (151.7 ng/dL) that rapidly increased over 6 weeks to 279.6 ng/dL. Ovarian tumor markers were within normal limits. After multidisciplinary review by Gynecology, Oncology, Surgery, and Radiology, and shared decision-making with the family, the patient underwent a laparoscopically-assisted laparotomy with right salpingo-oophorectomy due to concern for rapidly increasing testosterone levels in the tumor range. Intraoperative findings were notable for bilaterally enlarged ovaries (right greater than left), right paratubal cyst, and right adnexal torsion around the infundibulopelvic ligament. Pathological evaluation of the right ovary revealed a polycystic ovary with stromal edema and hyperthecosis; no evidence of a testosterone-secreting tumor or malignancy was present. Three weeks postoperatively, the total testosterone level decreased to 59.1 ng/dL.

Comments: In the setting of a large pelvic mass and virilization in an adolescent, ovarian hyperthecosis should remain on the differential in addition to virilizing ovarian tumors. OH causes bilaterally enlarged ovaries (5-7 cm in diameter) that can increase the risk for ovarian torsion and lead to asymmetric/unilateral adnexal enlargement due to stromal edema. While diagnosis can only be definitively determined with histopathologic examination, serum testosterone levels can aid in the diagnosis and in monitoring postoperatively on anti-androgen therapy.