Neoplastic potential in patients with disorders of sexual differentiation.

The occurrence of tumors was determined in 157 patients with disorders of sexual differentiation at a major pediatric hospital from 1960 to 1980. Gonadoblastoma developed in 2 of the 4 patients with mixed gonadal dysgenesis (MGD) who had not had bilateral gonadectomy; this indicates the need for early gonadectomy in such cases. Gonadal embryonal carcinoma developed in 1 child with Turner's syndrome (1 of 71). Four cases of nephroblastoma occurred, 1 in a child with MGD (1 of 18) and 3 in male pseudohermaphrodites (3 of 35). The nature of the defect linking male pseudohermaphroditism with Wilms tumor remains to be established.