Leydig cell tumors and tumors associated with congenital adrenal hyperplasia

Testicular cancers occur at a rate of 2 cases per 100,000 males. Gonadal stromal tumors, including Leydig cell tumors and tumors of the adrenogenital syndrome, account for 1% to 3% of these neoplasms. Despite their rarity, these hormone-producing tumors are particularly interesting because of their potential for causing endocrinologic manifestations in prepubertal and adult males. They are also clinically significant, and early identification is critical to avoid profound and often irreversible developmental changes in affected children. An accurate diagnosis is important to differentiate tumors that will respond to medical management from tumors that require definitive surgical therapy.