BIOSYNTHESIS OF STEROIDS BY TESTICULAR TUMORS COMPLICATING CONGENITAL ADRENOCORTICAL HYPERPLASIA*

Abstract

In vitro studies of bilateral testicular tumors occurring in a boy with congenital adrenocortical hyperplasia showed that these tumors synthesized large amounts of 17[alpha]-hydroxy-progesterone. The accumulation of 17[alpha]-hydroxyprogesterone in amounts greater than normal for either testicular or adrenal tissue is attributed to the low degree of activity of both 21-hydroxylase and side-chain splitting enzyme in the testicular tumors. The excretion of 17-ketosteroids was elevated in this patient. It is suggested that the excess urinary 17-ketosteroids were formed at some site other than the testicular tumors, from precursors which were part of the tumor secretion. This hypothesis supported by the low side-chain splitting activity found in the tumors, the increased levels of 17[alpha]-hydroxyprogesterone in spermatic vein blood after administration of ACTH, and the fall in both 17-ketosteroid and pregnanetriol excretion after bilateral orchidectomy.