Abstract
PURPOSE: Atypical teratoid/rhabdoid tumors (ATRTs) are very rare, highly malignant embryonal neoplasms in central nervous system. The aim of this study was to conduct a retrospective analysis of ATRT patient survival and investigate the prognostic factors associated with ATRT. METHODS: A retrospective study was conducted using information of patients who received treatment between 2016 and 2021 in Beijing Tiantan Hospital. Kaplan-Meier curves were used for overall survival (OS) analysis. Univariate and multivariate COX analyses were applied for OS predicting. RESULTS: 20 histologically confirmed ATRT patients were included. The majority were male (75%) and aged over 3 years (65%). 71.4% of patients under 3 years and 46.2% of above 3 years had supratentorial tumors. All patients underwent surgery, with 60% having total resections, primarily in the supratentorial region. Subsequent treatment involved varying chemotherapy and radiation combinations, with 40% of patients receiving it, and 87.5% of those were older than 3 years, The median overall survival for ATRT patients was 180 days. Survival differed significantly between patients under and above 3 years. Radiotherapy increased overall survival for all patients. Univariate and multivariate analysis showed better survival for those diagnosed above age 3 and with adjuvant radiation. CONCLUSIONS: Patients older than 3 years old had better prognosis and radiotherapy had a significant effect on improving patient prognosis.