PIK3CA and Beckwith-Wiedemann syndrome: Other syndromes associated with an elevated WT risk include Denys-Drash syndrome (DDS, WT1 point mutations) [11], and several overgrowth syndromes: Perlman (biallelic germline DIS3L2 mutation) [12], Simpson-Golabi-Behmel (GPC3/GPC4), PIK3CA-related overgrowth spectrum (PROS), and Beckwith-Wiedemann syndrome (BWS) [13].