TP53-Associated Pediatric Malignancies.

Although the majority of pediatric malignancies express wild-type p53, it is well established that germline TP53 mutations or functional inactivation of this pathway by other means contribute to childhood cancer. Epidemiology studies have revealed the existence of diverse inherited mutant TP53 alleles that display different levels of tumor suppressor activity, which correlate with cancer risk in terms of penetrance, age of onset, and tumor types. In this monograph, the authors describe those childhood cancers associated with functional inactivation of TP53 focusing on adrenocortical carcinoma as a model for tissues that are highly sensitive to loss of p53 activity.