Virilizing Adrenocortical Carcinoma

Abstract

CONGENITAL adrenal hyperplasia (CAH) is an uncommon disorder, but the incidence of virilizing adrenocortical neoplasms, benign and malignant, is even less common.¹The occurrence of both conditions in the same patient^2-5raises the question of whether or not the association is coincidental. The patient described herein was born with a salt-losing form of CAH, probably caused by a severe 21-hydroxylase deficiency; despite adequate control, a virilizing carcinoma of the adrenal cortex developed. <h3>Report of a Case</h3> An 11-year-old girl was admitted to the White Plains (NY) Hospital Medical Center because of progressive virilization associated with strikingly increasing levels of urinary 17-ketosteroids (17-KS). During the first week of life, the patient had had persistent vomiting, weakness, and convulsions. Serum sodium level had been 126 mEq/L, and serum potassium level, 9.2 mEq/L. The clitoris had been enlarged and the labia majora fused. A cystourethrogram had shown dye in a vaginal