Somatic mutations and deletions of WTX have been reported in 6 to 30% of patients with Wilms tumors (WT), a kidney cancer typical of childhood, arising from multipotent mesenchymal kidney precursors [7,44,46,49]; WTX has later been identified as OS-CS disease-causing-gene, both in familial and sporadic cases. Here, AMER1 is linked to Nephroblastoma.