Increasing hirsutism due to a granulosa-cell tumor in a woman with polycystic ovary syndrome: case report and review of the literature.

Abstract Background: Granulosa-cell tumors (GCT), rare malignancies that arise from sex-cord stromal cells, account for less than 5% of ovarian tumors. These tumors present with an endocrine syndrome and mass signs. Surgery is the primary treatment approach. The risk of recurrence is more frequent in the juvenile-onset form. Case report: We report the case of an obese 18-year-old Caucasian women with hirsutism and oligomenorrhea. Abdominal palpation revealed a voluminous firm mass. Hormonal evaluation documented severe hyperandrogenism. The ovary-specific tumor marker CA125 was elevated, whereas human-chorionic-gonadotropin was in the normal range. Abdominal imaging examination revealed a 19 cm mass in the left ovary. Twenty-four hours after removal of the mass, menstrual flow reappeared and androgens progressively normalized. Microscopically, the predominant pattern was one of uniform, bland, epithelioid to spindle-shaped cells. After three months, a significant weight loss was recorded, hirsutism had decreased slightly and oligomenorrhea reappeared. Δ4-Androstenedione levels remained elevated (4200 ng/L), whereas CA125 had normalized. In light of the pre-existing polycystic-ovary-syndrome (PCOS), the patient started estrogen-progestin treatment. Conclusion: We report an interesting case of a woman with severe hirsutism due to GCT, and a history of oligomenorrhea caused by PCOS. After surgery, a dramatic clinical improvement was observed, whereas PCOS signs persisted.