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Adrenal virilism is a syndrome in which excessive adrenal androgens cause virilization. Diagnosis is clinical and confirmed by elevated androgen levels with and without dexamethasone suppression; determining the cause may involve adrenal imaging. Treatment depends on the cause.
Adrenal virilism is caused by an androgen-secreting adrenal tumor or by adrenal hyperplasia. Malignant adrenal tumors may secrete excess androgens, cortisol, or mineralocorticoids (or all three), resulting in Cushing's syndrome (see Adrenal Disorders: Cushing's Syndrome) with suppression of ACTH secretion and atrophy of the contralateral adrenal as well as hypertension. Adrenal hyperplasia is usually congenital; delayed virilizing adrenal hyperplasia is a variant of congenital adrenal hyperplasia (see Endocrine Disorders in Children: Congenital Adrenal Hyperplasia). Both are caused by a defect in hydroxylation of cortisol precursors; cortisol precursors accumulate and are shunted into the production of androgens. The defect is only partial in delayed virilizing adrenal hyperplasia, so clinical disease may not develop until adulthood.
Symptoms and Signs
Effects depend on the patient's sex and age at onset and are more noticeable in women than in men. Symptoms and signs include hirsutism (sometimes the only sign in mild cases), baldness, acne, and deepening of the voice. Libido may increase. In prepubertal children, growth may accelerate. If untreated, premature epiphyseal closure and short stature occur. Affected prepubertal males may experience premature sexual maturation. Females may have amenorrhea, atrophy of the uterus, clitoral hypertrophy, decreased breast size, and increased muscularity. In adult men, the excess adrenal androgens may suppress gonadal function and cause infertility. Ectopic adrenal tissue in the testes may enlarge and simulate tumors.
Diagnosis
Adrenal virilism is suspected clinically, although mild hirsutism and virilization with hypomenorrhea and elevated plasma testosterone may also occur in polycystic ovary (Stein-Leventhal) syndrome (see Menstrual Abnormalities: Polycystic Ovary Syndrome). Adrenal virilism is confirmed by showing elevated levels of adrenal androgens. In adrenal hyperplasia, urinary dehydroepiandrosterone (DHEA) and its sulfate (DHEAS) are elevated, pregnanetriol excretion is often increased, and urinary free cortisol is normal or diminished. Plasma DHEA, DHEAS, 17-hydroxyprogesterone, testosterone, and androstenedione may be elevated. A level of > 30 nmol/L of 17-hydroxyprogesterone 30 min after administration of cosyntropin 0.25 mg IM strongly suggests the most common form of adrenal hyperplasia.
Virilizing tumors are excluded if dexamethasone 0.5 mg po q 6 h for 48 h suppresses production of excess androgens. If excessive androgen excretion is not suppressed, CT or MRI of the adrenals and ultrasonography of the ovaries are done to search for a tumor.
Treatment
Recommended treatment for adrenal hyperplasia is dexamethasone 0.5 to 1 mg po at bedtime, but even these small doses may cause signs of Cushing's syndrome. Cortisol 25 mg po once/day or prednisone 5 to 10 mg po once/day can be used instead. Although most symptoms and signs of virilism disappear, hirsutism and baldness disappear slowly, the voice may remain deep, and fertility may be impaired.
Tumors require adrenalectomy. For patients with cortisol-secreting tumors, hydrocortisone should be given preoperatively and postoperatively because their nontumerous adrenal cortex will be atrophic and suppressed.
Last full review/revision November 2007 by Ashley B. Grossman, MD
Content last modified November 2007
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