Virilizing syndrome associated with an adrenal cortical adenoma secreting predominantly testosterone

A 22 year old woman had virilization and mild Cushing's syndrome. Serum testosterone levels were high (3.2 ng/ml), urinary 17-ketosteroid levels slightly elevated (17.5 mg/d) and plasma cortisol at 16:00 hours normal (7.2 μg/dl). Basal level of cortisol secretion was normal but with evidence of autonomy. An abnormal adrenal scan led to an adrenal venogram showing a tumor image in the left adrenal gland. Selective adrenal venous catheterization indicated that the tumor was secreting testosterone and cortisol; left/right adrenal venous values were 53.5 3.7 ng/ml for testosterone and 92.8 13.6 μg/dl for cortisol. Testosterone levels did not rise with human chorionic gonadotropin (hCG). The basal serum level of Δ 4 androstenedione was at the upper limit of normal and of 17 α-hydroxyprogesterone high. The patient underwent surgical removal of a well circumscribed, 19 g left adrenal adenoma. Values for serum testosterone, DHEA-S and 17β-estradiol (E 2) decreased markedly postoperatively. The in vivo biochemical findings were corroborated in vitro by incubation of tumor tissue slices and nontumorous adrenal cortex. The tumor was capable of secreting testosterone, cortisol, DHEA and E 2, but the capacity was greatest for testosterone, DHEA and E 2. Analysis of tissue homogenates also revealed the presence of testosterone, cortisol, DHEA and E 2. The pattern of steroid production suggests partial 21-hydroxylase deficiency and enhanced 17β-reductase activity.