Wang Zhiliang, Yang Jingchen, Liu Xing, Liu Wei
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Department of Neuropathology Center, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China.
Front Pediatr. 2025 Mar 6;13:1463510. doi: 10.3389/fped.2025.1463510. eCollection 2025.
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.
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.
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.
Patients older than 3 years old had better prognosis and radiotherapy had a significant effect on improving patient prognosis.
非典型畸胎样/横纹肌样瘤（ATRT）是中枢神经系统中非常罕见的高度恶性胚胎性肿瘤。本研究的目的是对ATRT患者的生存情况进行回顾性分析，并调查与ATRT相关的预后因素。
利用北京天坛医院2016年至2021年期间接受治疗的患者信息进行回顾性研究。采用Kaplan-Meier曲线进行总生存（OS）分析。应用单因素和多因素COX分析进行OS预测。
纳入20例经组织学确诊的ATRT患者。大多数为男性（75%），年龄超过3岁（65%）。3岁以下患者中有71.4%、3岁以上患者中有46.2%患有幕上肿瘤。所有患者均接受了手术，其中60%实现了全切，主要在幕上区域。后续治疗包括不同的化疗和放疗组合，40%的患者接受了治疗，其中87.5%的患者年龄超过3岁。ATRT患者的中位总生存期为180天。3岁以下和3岁以上患者的生存情况有显著差异。放疗提高了所有患者的总生存率。单因素和多因素分析显示，3岁以上确诊且接受辅助放疗的患者生存情况更好。
3岁以上患者预后较好，放疗对改善患者预后有显著作用。
