SAT-165 - Occult Ovarian Steroid Cell Tumor Presenting with Severe Hyperandrogenism Despite Negative Imaging

A 42-year-old woman with hypertension, hyperlipidemia, and prediabetes was referred to Endocrinology for markedly elevated testosterone. She had menarche at age 13 and initially had regular menstrual cycles. Over time, her cycles became irregular and briefly improved after placement of a Mirena intrauterine device (IUD). Her menses stopped 18 months before evaluation. She reported hot flashes, vaginal dryness, scalp hair loss, and increased terminal hair growth. She denied acne, galactorrhea, testosterone exposure, and infertility.

On examination, she had male pattern alopecia and course dark hair on her face, chest, upper arms and upper back without clitoromegaly. She did not have withdrawal bleeding after a progesterone challenge, which prompted further evaluation. Laboratory testing showed normal follicle-stimulating hormone, luteinizing hormone, prolactin, adrenocorticotropic hormone, cortisol, dehydroepiandrosterone, 17-hydroxyprogesterone, and estradiol. Testosterone was markedly elevated at 581 ng/dL (12-48 ng/dL) which was confirmed on multiple repeats, and androstenedione was elevated at 1.052 ng/mL (0.130-0.820 ng/mL). A dexamethasone suppression test was negative. Pelvic ultrasound, abdominal and pelvic computed tomography (CT), and pelvic magnetic resonance imaging (MRI) showed no adnexal or adrenal mass. She was started on spironolactone, but testosterone levels did not improve. She did not desire future fertility.

Given her persistent severe hyperandrogenism and high concern for occult ovarian malignancy, she was referred to Gynecology Oncology and underwent therapeutic bilateral salpingo-oopherectomy 5 months after her initial Endocrine evaluation. Pathology revealed a 2.5 cm steroid cell tumor (SCT) confined within the left ovary. Postoperatively, testosterone decreased to 12 ng/dL.

SCTs are rare sex-cord stromal tumors with literature reporting less than 1000 cases. They are frequently hormonally active with secretion of androgen hormones and their metabolites leading to symptoms including virilization, balding, hirsutism, acne, deepening of the voice and clitoromegaly. An androgen secreting tumor should be suspected in females with late and rapid onset of virilization. Smaller lesions may not be seen on CT or MRI, which has higher sensitivity. Persistent hyperandrogenism warrants consideration of surgical exploration and therapeutic oopherectomy for a definitive diagnosis and management. Patients require long term follow up as recurrence can happen years later.

*Unless otherwise noted, all abstracts presented at ENDO must not be released to the press or the public until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.*