American Urological Association - Testicular Tumors of Adrenogenital Syndrome (TTAG)
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.