DNA damage response and its clinical implications in pediatric cancers

DNA damage response (DDR) is a complex network of biological pathways, maintaining eukaryotic genetic stability and frequently altered in cancer cells. Aberrant DDR regulation could be a double-edge sword in cancer: DDR defects could lead to genetic instability driving the acquisition of cancer mutations, while alternative DDR pathways could provide the survival benefits for genetic-unstable cancer cells. Targeting DDR defects in cancer, most noticeably through PARP inhibitors, exhibit impressive clinical efficacy in multiple cancer types. Here, we update recent progress concerning DDR and its inhibitors in pediatric cancers, from molecular mechanism to clinical practice.