Testicular adrenal rest tumors in a patient with congenital adrenal hyperplasia.

Congenital adrenal hyperplasia refers to a group of autosomal recessive disorders caused by a deficiency of an enzyme involved in the synthesis of glucocorticoids. The enzyme deficiency generally leads to a deficiency of cortisol and/or aldosterone production within the adrenal cortex. The lack of glucocorticoids generally leads to elevated levels of plasma corticotropin (ACTH), which often results in adrenal hyperplasia. Testicular adrenal rest tumors may develop in males with congenital adrenal hyperplasia due to overstimulation of aberrant adrenal cells within the testes. Recognition of this disease entity is essential when evaluating young males with testicular masses.