Editorial: Recent Advances in Pediatric Cancer Predisposition Syndromes

Cancer predisposition syndromes (CPSs) are an important cause of tumors in pediatric patients. Although a significant number of cancer predisposition genes have already been described, there are many pediatric patients with cancer in whom inherited cancer predisposition syndromes have yet to be detected. The prevalence of childhood cancer attributable to genetic predisposition is difficult to be estimated but recent reports suggest that at least 10% of pediatric cancer patients harbor a germline mutation in a cancer-predisposition gene. The advent of large-scale genome sequencing studies has profoundly helped our understanding of the biology of cancer predisposition, leading to better and earlier identification of individuals at high risk of cancer, selection of new molecular targets, and, in some cases, development of tailored approaches.