Bilateral testicular adrenal rests in a patient with 11-hydroxylase deficient congenital adrenal hyperplasia.

A 13-year-old boy was referred for investigation of hypertension, precocious puberty and giant testicles. Hormonal studies established the diagnosis of congenital adrenal hyperplasia due to 11-hydroxylase deficiency. Testicular biopsy revealed that the scrotal masses were adrenal rest tumors entirely composed of adrenocortical tissue. Primary testicular tissue was absent. The size of the tumors regressed following replacement steroid therapy together with restoration of normal blood pressure. This observation of a testicular adrenal rest tumor in a patient with 11-hydroxylase deficient congenital adrenal hyperplasia is unique.