True statement of wilms tumor

Refer Robbins 9/e 479 * The first group of patients has the WAGR syndrome, characterized by Wilms tumor, aniridia, genital anoma- lies, and mental retardation. Their lifetime risk of devel- oping Wilms tumor is approximately 33%. Individuals with WAGR syndrome carry constitutional (germline) deletions of 11p13. Studies on these patients led to the identification of the first Wilms tumor-associated gene, WT1, and a contiguously deleted autosomal dominant gene for aniridia, PAX6, both located on chromosome 11p13. Patients with deletions restricted to PAX6, with normal WT1 function, develop sporadic aniridia, but they are not at increased risk for Wilms tumors.