Female virilization

Specialty: Endocrine and metabolic.

• masculinization of women
• severe clinical hyperandrogenism
• androgenic virilization

Why it occurs

• Androgen-secreting ovarian tumor such as Sertoli-Leydig cell tumor, hilar cell tumor or thecoma (massive and autonomous production of testosterone)
• Androgen-producing adrenal tumor such as adrenocortical adenoma or carcinoma (excessive production of dehydroepiandrosterone sulfate and androstenedione)
• Non-classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency (partial enzymatic blockade that diverts steroid synthesis towards the androgenic pathway)
• Polycystic ovary syndrome severe phenotype with ovarian hyperandrogenism and profound hyperinsulinemia
• Exogenous exposure to anabolic steroids or androgens for sports or hormonal use

Initial workup

Total and free testosterone in serum; dehydroepiandrosterone sulfate (DHEA-S, adrenal androgenic marker); 17-hydroxyprogesterone at baseline and after stimulation with ACTH (to rule out congenital adrenal hyperplasia); androstenedione; high-resolution transvaginal ultrasound with color Doppler to examine the anatomy of the ovaries; and high-resolution computed tomography (CT) of the adrenal glands.

Standard management

• Spironolactone — androgen receptor antagonist and weak inhibitor of androgen synthesis, administered at doses of 100-200 mg/day, monitoring potassium levels
• Cyproterone acetate — synthetic progestogen with potent peripheral antiandrogenic activity, usually combined with ethinyl estradiol
• Finasteride — inhibitor of the 5-alpha-reductase type 2 enzyme to reduce the local cutaneous conversion of testosterone to dihydrotestosterone
• Dexamethasone at low doses — 0.25-0.5 mg at night in cases of non-classical congenital adrenal hyperplasia to suppress the secretion of ACTH and consequently adrenal androgens