American Urological Association - True Hermaphroditism
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True Hermaphroditism

  • Characterized by presence of testicular and ovarian tissue in same individual.
  • Rare and difficult to diagnose; should be suspected in all individuals with ambiguous sex characteristics.
  • Clinical: hermaphrodites raised as males come to attention at puberty when they develop breasts (95%) or have periodic hematuria; hermaphrodites raised as females come to attention for clitoromegaly or irregular menses.
  • Gross: gonads may be testes, ovaries, or ovotestes.
  • Histology:
    • Ovotestes are most common form, and contain both ovarian and testicular tissue; interstitium may contain Leydig cells. Ovaries may become functional at puberty but seminiferous tubules remain immature.
    • Ovaries are usually left-sided and contain primordial follicles; may be functionally and histologically normal (rare).
    • Testes are usually on the right side and may be intraabdominal or intrascrotal; complete spermatogenesis is rare.
  • These patients are at a high risk of malignancy, including gonadoblastoma, dysgerminoma/ seminoma, and yolk sac tumors.