Bahar Michal, Kordes Uwe, Tekautz Tanya, Wolff Johannes
Department of Pediatric Hematology Oncology and Blood and Marrow Transplantation, Cleveland Clinic, Cleveland, OH, U.S.A.
Department of Pediatric Hematology Oncology and Blood and Marrow Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Anticancer Res. 2015 May;35(5):3013-7.
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.
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.
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).
This finding provides evidence for pursuing treatment approaches that do not include radiation therapy for patients with LFS.
脉络丛癌（CPCs）是一种罕见的儿科肿瘤，常与李-佛美尼综合征（LFS）相关，LFS是一种TP53肿瘤抑制基因的种系突变，易患癌症。
我们对1990年至2013年的文献进行了系统回顾，以评估CPC和LFS患者应避免放疗这一假设。使用Kaplan-Meier曲线和对数秩检验比较总生存期（OS）。
记录了28例CPC和LFS患者。17例患者中有11例接受了放疗。接受放疗的患者的生存期低于未接受放疗的患者[中位（±9５%置信区间）2年总生存率=0.18±0.12%对0.58±0.12%]。对数秩检验表明差异具有边缘显著性（p=0.056）。
这一发现为寻求不包括放疗的治疗方法提供证据，用于LFS患者。
