Institute: Biocenter, Chair of Developmental Biochemistry, University of Würzburg, Germany
Wilms tumors, also called nephroblastoma, helped to establish the paradigm of the two-hit hypothesis of tumor genetics that shaped the concept of tumor suppressor genes. Over the years, genetic analysis has uncovered a plethora of genetic changes that appear to be capable of inducing such tumors, but there is little correspondence between genetics and the rather unusually spectrum of histological appearances. Analysis of further, even less frequent types of pediatric kidney cancers revealed additional, private sets of genetic drivers. Intriguingly, many of the culprit genes do not play a major role in adult cancer. Together with the striking differentiation potential that is observed in many of these tumors, this suggests that pediatric kidney cancers rather represent a case of aborted or misguided development.