Wilms Tumor Genetics: A New, UnX-pected Twist to the Story

The study of the genetics of Wilms tumor has led to several highly unexpected and precedent-establishing discoveries. Ironically, however, the identification of “WT genes” has been painfully slow, and gene mutations have been identified in only ∼25% of tumors. The discovery of an X chromosome gene, WTX, that is mutated somatically in ∼30% of Wilms tumors is notable both for helping to explain the genetic etiology of a substantial proportion of tumors and also for underscoring the role that X chromosome genes can play in cancer genetics.