Abstract
Recent progress in the study of the genetic basis of cancer, such as genomic stability, DNA repair and cell-cycle control, is changing our insight into how heredity contributes to the risk of childhood cancer. Although familial cancer in children is rare, the role of inherited genes in the etiology of childhood neoplasia needs to be reevaluated. Our progress in understanding the genetic factors predisposing individuals to familial cancer might help to understand the genetic basis of sporadic cases of childhood neoplasia. Recent progress in the study of genetics and hereditary factors of childhood neoplasia is reviewed and discussed.