Testicular Tumors of Adrenogenital Syndrome (TTAG)
Rare testicular growth of adrenal-like cells and resembles Leydig cell tumor that occurs in patients with congenital adrenal hyperplasia.
Probably a non-neoplastic growth.
Most commonly due to 21-hydroxylase deficiency.
Involvement is bilateral and frequently multiple, commonly at testicular hilus.
Most patients present with bilateral testicular pain or masses.
Gross: well-circumscribed, unencapsulated, solid and lobulated brown tumor.
Nodules, nests or cords of large cells with abundant pink cytoplasm (resemble Leydig cell), separated by fibrous tissue (image A) & (image B).
"High-grade" appearing nuclei with frequent nuclear pleomorphism but absent mitosis.
Lipofuscin pigment may be abundant.
Reinke crystalloids are absent.
Benign prognosis. (Despite atypical nuclei with pleomorphism)
Leydig cell tumor: usually painless, unilateral, most lacks nuclear pleomorphism, and a third has Reinke crystalloid.
Leydig cell hyperplasia: multiple small (<0.5 cm) nodules.