Accordingly, it has been reported that hypermethylation at H19/IGF2:IG-DMR is present in the premalignant clonal expansions of WTs, representing an early event in Wilms tumorigenesis [21,22], and as constitutional epimutation in the Beckwith-Wiedemann syndrome cases with high predisposition to WTs [5,23,24,25,26]. This evidence concerns the gene H19 and Beckwith-Wiedemann syndrome.