Wilms Tumor and WAGR syndrome
The “W” in WAGR syndrome stands for Wilms tumor. Approximately 50% of children with WAGR syndrome will develop Wilms tumor. Periodic screening should begin at birth or upon diagnosis of WAGR syndrome. Screening for Wilms tumor consists of abdominal ultrasound examinations every 3 months until age 8. Some physicians also advocate training parents to examine the child’s abdomen between ultrasounds.
Wilms tumor in WAGR syndrome tends to occur at an earlier age than in typical children. The average age of diagnosis of Wilms tumor in children with WAGR syndrome is 17-28 months. Most cases of Wilms tumor in children with WAGR syndrome are diagnosed by age 3. In rare cases, Wilms tumor has been reported in individuals with WAGR syndrome up to age 25. For this reason, some form of monitoring should be continued throughout life, such as ultrasound examinations every 6 to 12 months, and/or observing the child/adult for potential symptoms listed below.