Wilms' tumor in seven children with congenital aniridia.

This report reviews our experience with 26 infants and children who demonstrate the syndrome of congenital aniridia. Twenty patients exhibited congenital sporadic aniridia, and unilateral Wilms' tumor has developed in seven of them. Six of the total of 26 patients had familial aniridia and none has shown evidence of the development of a Wilms' tumor or any other malignant tumor to date. Fraumeni studied 15 patients with congenital sporadic aniridia. Five developed Wilms' tumors. In the combined series of 35 patients with congenital sporadic aniridia, 12 patients have developed Wilms' tumor for an incidence of 34%. A brief review of our experience and a suggested protocol for the evaluation of patients with congenital aniridia is presented.