Recent studies have highlighted the role of genetic alterations that play a key role in nephroblastoma pathogenesis, in particular, mutations in key regulatory genes such as WT1, WT2, CTNNB1, WTX, and TP53, which are associated with the development and progression of nephroblastoma tumors [96,99,126,127]. This evidence concerns the gene CTNNB1 and Wilms tumor.