Abstract
Most pediatric specialists, including hematologists/oncologists, surgeons, radiologists, and pathologists, are familiar with the diagnosis and management of Wilms tumor (WT). However, it may be challenging to identify the underlying conditions causing cancer predisposition, which can change the management for the patient and potentially their entire family. In this paper, we present 3 cases of clinically suspected WT associated with Beckwith-Wiedemann syndrome (BWS). We review the radiologic and histologic findings to diagnose BWS. We also discuss the implications of a BWS diagnosis on the clinical management of WT and follow-up guidelines for BWS patients.