Received:05 November 2025,
Revised:2026-03-23,
Accepted:30 March 2026,
Published:01 July 2026
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.Guideline for the diagnosis and treatment of childhood atypical teratoid/rhabdoid tumor of the central nervous system (2026 edition)[J].Journal of Chongqing Medical University,
.Guideline for the diagnosis and treatment of childhood atypical teratoid/rhabdoid tumor of the central nervous system (2026 edition)[J].Journal of Chongqing Medical University, DOI:10.13406/j.cnki.cyxb..
儿童中枢神经系统非典型畸胎样/横纹肌样瘤是一种罕见的高度恶性胚胎性肿瘤,好发于3岁以下儿童。该病发病率低,约占儿童脑肿瘤的1%~2%,但恶性程度高、进展迅速,临床表现多样,部分患者在确诊时已发生转移,早期诊断困难。目前其分子分型仍在不断更新,但缺乏统一的分期系统和标准治疗方案。本指南基于国内外最新临床研究证据、专家共识及实践经验制定,旨在对儿童中枢神经系统(central nervous system,CNS)非典型畸胎样/横纹肌样瘤(atypical teratoid/rhabdoid tumor,AT/RT)诊疗过程中的常见临床问题进行规范,为临床医护人员提供循证医学依据。
Childhood atypical teratoid/rhabdoid tumor(AT/RT) of the central nervous system(CNS) is a rare and highly malignant embryonal neoplasm and mainly affects children under 3 years of age. This disease has a low incidence rate and accounts for 1%-2% of childhood brain tumors; however,due to its high malignancy,rapid progression,and diverse clinical manifestations,some patients have experienced metastasis at the time of confirmed diagnosis,posing significant challenges for early diagnosis. At present,the molecular subtyping of this disease continues to evolve,and there is still a lack of a unified staging system and standardized treatment regimens. This guideline is developed based on the latest clinical research evidence,expert consensus,and practical experience in China and globally,and it aims to standardize common clinical issues encountered in the diagnosis and treatment of childhood AT/RT of the CNS,in order to provide evidence-based medical guidance for healthcare professionals.
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