MORE THAN MEETS THE EYE IN A PATIENT WITH PCOS: ANDROGEN-SECRETING GRANULOSA CELL OVARIAN TUMOR IN A VIRILIZED WOMAN WITH POLYCYSTIC OVARIAN SYNDROME (PCOS)

Abstract

Objective: Granulosa cell ovarian tumors are known to secrete estrogen. Herein we report a patient presenting with primary amenorrhea and virilization with markedly high androgen levels, all thought to be disproportional to be attributed to polycystic ovary syndrome (PCOS) alone. Bilateral oophorectomy revealed a rare androgen-secreting granulosa cell ovarian tumor and bilateral cysts (PCOS) both contributing to manifestations. Methods: Description of a case and discussion of the literature. Results: A 25-year-old female presented with primary amenorrhea, male pattern baldness and hirsutism of the face and chest, clitoral hypertrophy, cystic acne on the chest and shoulders, and type 2 diabetes. Diagnosis of PCOS was made at age 13 years. The concurrent presence of congenital adrenal hyperplasia was also considered. She was receiving metformin, oral contraceptives, and/or spironolactone with no improvement in manifestations at presentation to the endocrine clinic. She reported several elevated serum testosterone and androstenedione levels. Diagnosis of PCOS was established by the presence of enlarged cystic ovaries on ultrasound examination. The patient reported worsening manifestations including progression of diabetes despite therapy with metformin. She shaved her face, chest, and breasts daily for cosmetic reasons. Laboratory testing demonstrated markedly elevated total testosterone level (234 ng/dL), and normal cortisol and adrenocorticotropic hormone levels. Dexamethasone suppression and human chorionic gonadotropin stimulation indicated ovaries as a probable source of excessive circulating androgen levels. A computed tomography scan of the abdomen and pelvis showed multiple cysts in both ovaries, the largest being 1.9 cm in the right ovary. Due to the severity of manifestations, the patient was counseled to undergo fluoroscopically guided blood sampling of bilateral adrenal and ovarian veins, which confirmed both ovaries to be the source of the excess androgen production with a greater contribution by the right ovary. The patient underwent bilateral oophorectomy. Pathologic examination confirmed the presence of bilateral polycystic ovaries as well as an androgen-secreting granulosa cell tumor in the right ovary which apparently contributed to greater androgen levels in a right ovarian venous blood sample. Peripheral venous androgen levels normalized promptly after bilateral oophorectomy. Gradual resolution of clinical manifestations followed. Conclusion: A unique presentation of granulosa cell ovarian tumor with concurrent PCOS contributing to the extremely excessive production of androgens in a young woman manifesting primary amenorrhea and masculinization at the onset of puberty with marked gradual resolution of manifestations following bilateral oophorectomy.