Coincidental diagnosis of an occult hilar steroid cell tumor of the ovary and a cortisol-secreting adrenal adenoma in a 49-year-old woman with severe hyperandrogenism.

Objective: To report an exceptional association between an occult ovarian steroid cell tumor and a cortisol-secreting adrenal adenoma.

Methods: Case report. Methods: Endocrinology and nutrition unit at a general hospital in Spain. Methods: A 49-year-old woman who presented with persistence of severe hyperandrogenism after removal of a left adrenal adenoma. Methods: An endocrine study evaluating serum androgens, adrenal function, and tumor markers was ordered. Transvaginal sonography was done to rule out an ovarian tumor. Finally, a selective catheterization of ovarian veins allowed the correct diagnosis. Methods: Clinical and endocrine description of the patient and preoperative localization of the source of T secretion.

Results: After adrenal surgery, urinary free cortisol levels decreased to normal values, but serum T remained within the tumoral range (3.04 ng/mL). Selective catheterization of ovarian veins revealed a gradient of T concentration in the right ovary. After bilateral annexectomy, a microscopic steroid cell tumor of hilar type was found in the right ovary. Serum T fell within the normal range, and hirsutism progressively improved.

Conclusions: This unusual association between an occult-virilizing ovarian steroid cell tumor and a cortisol-secreting adrenal adenoma illustrates the value of an accurate preoperative workup in women with severe hyperandrogenism.