title: Radiation therapy for choroid plexus carcinoma patients with Li-Fraumeni syndrome: advantageous or detrimental?
abstract: BACKGROUND
  Choroid plexus carcinomas (CPCs) are rare pediatric tumors often associated with Li-Fraumeni Syndrome (LFS), a germline mutation in the TP53 tumor-suppressor gene, predisposing to cancer.
  
  
  METHODS
  We performed a systemic literature review from 1990-2013 to evaluate the hypothesis that radiation therapy should be avoided in patients with CPC and LFS. Overall survival (OS) was compared using Kaplan-Meier curves and log-rank tests.
  
  
  RESULTS
  Twenty-eight patients were documented with CPC and LFS. Eleven out of 17 patients received radiation therapy. The survival of patients receiving radiation was inferior to that of those without radiation [median (±95% confidence interval) 2-year OS=0.18 ± 0.12% versus 0.58 ± 0.12%]. The log-rank tests suggested the difference to be marginally significant (p=0.056).
  
  
  CONCLUSIONS
  This finding provides evidence for pursuing treatment approaches that do not include radiation therapy for patients with LFS.
authors.affiliation: Department of Pediatric Hematology Oncology and Blood and Marrow Transplantation, Cleveland Clinic, Cleveland, OH, U.S.A. baharm@ccf.org.
authors.forename: Michal
authors.initials: M
authors.lastname: Bahar
authors.affiliation: Department of Pediatric Hematology Oncology and Blood and Marrow Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors.forename: Uwe
authors.initials: U
authors.lastname: Kordes
authors.affiliation: Department of Pediatric Hematology Oncology and Blood and Marrow Transplantation, Cleveland Clinic, Cleveland, OH, U.S.A.
authors.forename: Tanya
authors.initials: T
authors.lastname: Tekautz
authors.affiliation: Department of Pediatric Hematology Oncology and Blood and Marrow Transplantation, Cleveland Clinic, Cleveland, OH, U.S.A.
authors.forename: Johannes
authors.initials: J
authors.lastname: Wolff
mesh_terms: D002277:Carcinoma / Q000150:complications / Q000473:pathology / Q000532:radiotherapy*; D002648:Child; D002675:Child, Preschool; D016545:Choroid Plexus Neoplasms / Q000150:complications / Q000473:pathology / Q000532:radiotherapy*; D005260:Female; D020022:Genetic Predisposition to Disease; D018095:Germ-Line Mutation; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D016864:Li-Fraumeni Syndrome / Q000150:complications / Q000473:pathology / Q000532:radiotherapy*; D008297:Male; D016159:Tumor Suppressor Protein p53 / Q000235:genetics*
chemical_list: D016159:Tumor Suppressor Protein p53
keywords: Li-Fraumeni syndrome; TP53 mutation; choroid plexus carcinoma; radiation