Survival Benefit from Multimodal Treatment for Patients with Atypical Teratoid Rhabdoid Tumor in a Surveillance, Epidemiology and End Result Database Analysis

Introduction Atypical teratoid rhabdoid tumor (ATRT) is among the most aggressive central nervous system malignancies. Although rare, this tumor typically afflicts young children and results in mortality within months. Here, we aim to determine key clinical features and treatment options that impact survival of patients with ATRT. Methods From the years 2000 to 2018, 363 patients with ATRT were identified from the Surveillance, Epidemiology, and End Results database. Univariate analysis was used to identify variables that had a significant impact on the primary endpoint of overall survival (OS). Multivariable analysis was then used to identify independent predictors of survival.Results Median OS of the entire cohort was 13 months. Univariate analysis identified ages between 1-3 years, ages between 4-17 years, years of diagnosis between 2010-2019, and receipt of treatment to have a significant impact on survival. In multivariable analysis, ages between 1-3 years and receipt of treatment were the only significant independent predictors of survival. Median OS was significantly greater in patients that received surgical treatment, chemotherapy, or radiation when compared to those that did not receive any treatment. In general, receipt of any combination of therapies improved median OS significantly. The receipt of triple therapy had the greatest impact on survival.Discussion/Conclusion This study highlights the survival benefit of a multimodal approach in the treatment of ATRT. The use of triple therapy, including surgery, radiation, and chemotherapy, was found to have the greatest survival benefit for patients. Overall, these findings may guide future care for patients with ATRT.