Virilization syndrome in a young woman due to an androgen-secreting adenoma

Abstract

A virilization syndrome in a normally menstruated 20--year-old woman, due to adrenocortical tumor, is reported. Preoperative basal hormonal study showed normal plasma gonadotropins, androstenedione (A) and cortisol, slightly elevated testosterone (T), markedly increased urinary 17 ketosteroids (17KS) excretion and plasma dehydroepiandrosterone-sulfate (DHEA-S) levels. Thus DHEA-S was the major constituent of the androgen excess. Urinary 17KS and plasma DHEA-S did not change after ACTH, whereas increased after dexamethasone (DXM)-hCG administration. PRL and LH responses to TRH and LHRH tests were exaggerated. Echotomography, pneumoperitoneum radiography and selective adrenal venous angiography showed a tumor in the left adrenal gland. After the tumor removal all abnormalities were corrected. Light and electron microscopy showed well differentiation of the tumoral cells with typical ultrastructural characteristics of androgen-secreting tissue. It is concluded that the absence of amenorrhea and of LH suppression does not exclude the possibility of an androgen-secreting tumor. Moreover, the unusual finding of slightly elevated T value may be reported to enzymatic defect at the neoplastic cellular level. Furthermore the paradoxical DXM-hCG androgen response may be due to spontaneous variations in hormone secretion by the tumor or to cell receptors alteration.