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.
D000293:Adolescent; D000328:Adult; D002648:Child; D012734:Disorders of Sex Development / Q000150:complications* / Q000235:genetics; D004407:Dysgerminoma / Q000209:etiology*; D005260:Female; D006801:Humans; D007621:Karyotyping; D007680:Kidney Neoplasms / Q000209:etiology*; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000209:etiology*; D010051:Ovarian Neoplasms / Q000209:etiology*; D012306:Risk; D013736:Testicular Neoplasms / Q000209:etiology*; D009396:Wilms Tumor / Q000209:etiology*