BILATERAL TESTICULAR TUMORS COMPLICATING CONGENITAL ADRENOCORTICAL HYPERPLASIA

Abstract

A case of bilateral steroid-secreting testicular tumors in a boy with congenital adrenocortical hyperplasia of the salt-losing type is presented. The steroidogenic activity of the testicular masses in vitro resembled that of abnormal adrenocortical tissue. Although large amounts of 17-ketosteroids were present in the urine, studies of the spermatic vein blood and testicular biosynthetic activity indicated that the tumors produced precursors for 17-ketosteroids but not the 17-ketosteroids themselves. There is evidence that endogenous ACTH played a role in tumor growth and secretion in this patient. It was not possible, from the studies performed, to determine whether the testicular tumors were derived from adrenocortical rest cells or from testicular interstitial cells. The importance of therapy with exogenous steroids aimed at suppressing endogenous ACTH production and urinary 17-ket6steroids is stressed.