[Li-Fraumeni Syndrome-Current Status and Prospects in Clinical Practice].

: Li-Fraumeni syndrome(LFS)is a cancer predisposing disorder with early-onset cancers in diverse tissues of origin, caused by germline TP53 mutation. In LFS, 5 core cancers are known: breast cancer, soft tissue sarcoma, osteosarcoma, brain tumor, adrenocortical cancer. Surveillance for LFS must be lifetime long and multitargeted since the patients are susceptible to various cancers from infant to middle-aged and older. Recent investigations have revealed germline TP53 mutations can increase the riskfor radio-induced tumorigenesis and will affect patient's prognosis and treatment in several cancers. The surveillance protocol has developed in Canada and several prospective studies based on the Canadian's one are currently being carried out. We are aware that the surveillance protocol suitable for the current status in Japan is urgently needed to provide the appropriate medical care for LFS patients.