The effectiveness of Wilms tumor screening in Beckwith–Wiedemann spectrum

Purpose: It is well documented that patients with Beckwith–Wiedemann spectrum (BWS) have a significantly higher risk of developing Wilms tumor (WT) than the general population. There has been little research on the timing of WT diagnosis in BWS in regard to optimizing suggested screening protocols. Methods: A literature search was performed to identify all reports of patients with BWS and WT. These data were combined with unpublished data from patients in the authors’ cohorts. Age at WT diagnosis was compared against data collected through the NIH Surveillance, Epidemiology, and End Results Program (SEER) registry. Results: Patients with BWS had a significantly higher incidence of WT diagnoses between age 12 and 84 months compared to patients in the SEER registry. Patients with BWS and WT diagnosed through screening had significantly lower stages at diagnosis compared to patients with BWS that were not screened. Conclusions: Screening until age 7 years is effective in detecting close to 95% of all WT in patients with BWS.