Abstract

BackgroundThe role of postoperative radiotherapy (PORT) in children with primary intracranial atypical teratoid/rhabdoid tumor (AT/RT) remains controversial, and real-world data with large sample size are still lacking. This study aims to estimate the survival benefit of PORT in pediatric patients with resected AT/RT. MethodsUsing Seer database, we collected 246 eligible intracranial AT/RT patients diagnosed between 2000 and 2016 for our analysis. Propensity score matching (PSM) analysis was employed to minimize selection bias for evaluation of the PORT efficacy. Multivariate Cox regression was conducted to determine the factors related to the outcome. Interaction tests were further performed between PORT and the prognostic variables. After identifying the significant prognostic factors, we further developed a novel prediction model to predict the life expectancy of these patients, as well as the potential benefit from PORT. ResultsWe found that PORT was significantly related to the improved survival after adjusting for other prognosticators in both the entire and PSM-matched cohort. Significant interactions of PORT with age at diagnosis and tumor extension were also observed. On basis of the prognostic indictors identified by L1-penalized lasso Cox regression analysis, a novel nomogram model was successfully established and externally validated. ConclusionOur study indicated that PORT was significantly associated with the improved survival in pediatric AT/RT patients, and the greater survival benefit from PORT could be achieved in patients <3 years old or with locoregional tumors. The novel prediction model was developed to provide help in clinical practice and in the design of related trials.

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