IGF2 and Wilms tumor: Perlman syndrome is an autosomal recessive syndrome due to mutation in the gene encoding DIS3L2 exoribonuclease (RNAase); DIS3L2 has been shown to lead to IGF2 overexpression, as seen in the Beckwith–Wiedemann syndrome and is strongly associated with tissue overgrowth and Wilms tumor development (84).