The case of the elusive androgen

Objective: To describe a case of androgen excess and discuss the important factors in diagnosis and management. Methods: A case report is presented of a postmenopausal woman who had had severe hirsutism for 18 months. Her history, clinical and laboratory findings, treatment, and outcome are chronicled. The pertinent literature-especially that related to the differential diagnosis of hyperandrogenism-is also reviewed. Results: A 62-year-old woman had progressive hirsutism of the face, back, and abdomen as well as alopecia of the scalp, for which spironolactone therapy had proved ineffective. Laboratory studies showed a testosterone level of 644 ng/dL. Preoperative evaluation pointed to an ovarian source of testosterone. After total abdominal hysterectomy and bilateral oophorectomy, histologic examination of the ovaries showed bilateral hilar cell hyperplasia. Three months later, the serum testosterone level remained high (556 ng/dL), and repeated computed tomography of the abdomen disclosed a previously unseen 9-mm adenoma of the left adrenal gland, which was removed laparoscopically. Because of a persistently high testosterone value (546 ng/dL), the patient underwent dexamethasone suppression studies, followed by adrenal stimulation with corticotropin; no pathologic findings were demonstrated. Finally, gonadotropin suppression with nafarelin, 200 μg intranasally daily for 6 weeks, yielded a prompt and sustainable decrease in the testosterone level. This result was associated with dramatic clinical improvement. Conclusion: It is speculated that the patient had residual testosterone-producing tissue originating from primitive mesenchymal cells from the urogenital ridge, which was responsive to gonadotropins, in an unidentified abdominal or pelvic site.