TESTICULAR ENLARGEMENT IN PATIENTS WITH 11-HYDROXYLASE DEFICIENCY

Testicular nodules or tumors have been well described in patients with congenital adrenal hyperplasia (CAH) and usually associated with 21-h ydroxylase deficiency. The authors report on a Il-hydroxylase-deficien t patient presenting bilateral testicular enlargement and review the l iterature. Testicular biopsy was not very helpful to make differential diagnosis between adrenal rest hyperplasia and Leydig cell tumor. The size of testes regressed after steroid replacement therapy, and this observation was suggestive for adrenal rest hyperplasia. These finding s suggest that bilateral testicular enlargement in patients with CAH m ay occur after excessive adrenocorticotrophic hormone stimulation of c ells differentiated from unknown origin. Orchiectomy is not required b ut bilateral testicular biopsy must be performed. Copyright (C) 1997 b y W.B. Saunders Company.