Association of radiotherapy use and time to second cancer in children with Li Fraumeni syndrome

Li Fraumeni syndome (LFS) arising from germline TP53 mutation results in defective DNA repair and increased risk of multiple prlmary cancers beginning in childhood. Curative intent radiotherapy is often used to treat childhood cancer but its impact in children with LFS has not been reviewed. A retrospective case-series review of 4 LFS children with a solid cancer diagnosed before age 17 years at the University of Manchester (UK) assessed time to and survival following second cancer diagnosis. Following radiotherapy for the first cancer diagnosis, median time to seconde primary cancer diagnosis was 13.3 years and median survival 9.7 years. When no radiotherapy was used, median time to second primary cancer diagnosis was 25.1 years (Chi²=14.8; p<0.0001; HR 7.9) and median survival was 29.2 years (Chi²=12.5; p=0.004; HR 3.2). The authorx conclude that radiotherapy for first cancer in children with LFS is associated with adverse outcomes and ought be considered only in the absence of other potentially curative options.