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December 17, 1982

Virilizing Adrenocortical CarcinomaDevelopment in a Patient With Salt-Losing Congenital Adrenal Hyperplasia

JAMA. 1982;248(23):3140-3141. doi:10.1001/jama.1982.03330230052032

CONGENITAL adrenal hyperplasia (CAH) is an uncommon disorder, but the incidence of virilizing adrenocortical neoplasms, benign and malignant, is even less common.1 The occurrence of both conditions in the same patient2-5 raises 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.

Report of a Case  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