Predisposition Footprints in the Somatic Genome of Wilms tumour

Approximately 10% of children with cancer harbour a mutation in a predisposition gene. In children with the kidney cancer Wilms tumour, the prevalence is as high as 30%. Certain predispositions are associated with defined histological and clinical features, suggesting differences in tumorigenesis. This cohort study found that tumour development differed in children harbouring a predisposition, depending on the variant gene and its developmental timing. Treatment may therefore require a variant-specific approach. Overall, findings showed that certain predispositions dictate the genetic development of tumours, with clinical implications for these children.