ODP622 A Case of a Rare Steroid Cell Tumor in a Patient with PCOS

BACKGROUND: Ovarian sex cord-stromal tumors are rare and represent approximately 7% of all primary ovarian tumors. Steroid cell tumors of the ovary are uncommon sex-hormone secreting tumors and are classified as pure stromal tumors. These tumors are known to secrete hormones like androstenedione, alpha-hydroxyprogesterone, and testosterone. Polycystic Ovarian Syndrome (PCOS), a disorder affecting 6% to 10%, is characterized by hyperandrogenism and ovulatory dysfunction. We report a case of a patient with known PCOS who was found to have steroid secreting tumor. CLINICAL CASE: A 35 years old female presented to our office complaining of worsening hirsutism, amenorrhea and weight gain. Her past medical history was significant for PCOS, hypothyroidism, and obesity. Physical Examination was notable for hirsutism, obesity and clitoromegaly. Initial labs showed elevated testosterone level of 134 ng/dL (normal levels 0-70 ng/dL). Other labs ruled out congenital adrenal hyperplasia (CAH). Pelvic US was consistent with known PCOS but did not revealed additional findings. Endometrial biopsy was unremarkable. Subsequent labs showed continuous significant elevation in testosterone to 382 ng/dL with free testosterone level of 24 ng/dL. CT abdomen and pelvis with and without contrast showed a right solid adnexal mass. Patient underwent staging laparotomy with bilateral salpingo-oophorectomy. Pathology result was significant for well differentiated sex cord-stromal tumor, steroid secreting type. Genetic testing came positive for BRCA 1. CONCLUSION: This case illustrates that in the setting of rapid onset of hirsutism and elevated testosterone, an androgen secreting ovarian tumor should be considered and ruled out, even in the setting of known PCOS. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.