ADRENAL CORTICAL HYPERPLASIA WITH VIRILISM: DIAGNOSIS, COURSE AND TREATMENT 12

Abstract

The total urinary 17-ketosteroid excretion is abnormally increased in patients with adrenal cortical hyperplasia or adrenal cortical carcinoma. However, the magnitude of the total 17-ketosteroid excretion is not always a reliable index of the exact nature of this hyperactivity. The urinary output of the [beta]-alcoholic and the non-alcoholic 17-keto-steroids was markedly elevated in patients with adrenal cortical carcinoma, whereas their output was either normal or but slightly elevated in patients with cortical hyperplasia. Assay of the excretion of these latter 2 fractions therefore is of diagnostic value in differentiating patients with adrenal cortical hyperplasia from those with carcinoma of the adrenal cortex. The urinary pregnanediol values are not consistently elevated in patients with adrenal cortical hyperplasia. Adrenal cortical virilism, when due to hyperplasia of the adrenal cortex, usually persists without a tendency to spontaneous remissions. Children of both sexes may tend to grow rapidly in the early stages of the disease, but cease growing at an abnormally early age because of premature closure of the epiphyses. There is a probability that the boys will become normal appearing adult [male][male] but whether or not they will attain fertility is uncertain. If left untreated, the [female][female] tend to remain chronically masculinized. The treatment of choice for patients with a carcinoma of the adrenal cortex is surgical removal. Surgery is not indicated in the treatment of patients with adrenal cortical hyperplasia. In girls with this latter disease, orally administered diethylstilbestrol causes development of the [female] secondary sexual characteristics and improvement in the psychological outlook. There appears to be little indication for such therapy in the [male].