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.
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.