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

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Image B

  • 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.
  • Histology:
    • 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)
  • DDX:
    • 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.