ZFTA融合室管膜瘤的诊治研究进展

Diagnosis,treatment and prognosis of ZFTA fusion ependymomas

室管膜瘤包括多种类型,可发生在幕上腔室、颅后窝或脊髓。世界卫生组织(WHO)根据特定的解剖部位、组织学、基因表型的特征,对室管膜肿瘤分类系统进行了多次更新,并应用新的分类系统来预测评估病人的治疗结果。2021年,WHO组织更新分类,因为ZFTA融合阳性室管膜瘤的基因融合不仅涉及RELA,还包含如MAML2/3、NCOA1/2、MN1或CTNNA2等基因融合,所以室管膜瘤ZFTA融合阳性型成为了最新的室管膜瘤类型。本文通过回顾幕上室管膜瘤分类的变化、临床特点及治疗进展,概述ZFTA融合室管膜瘤的临床表现与治疗策略选择方面的研究进展,希望能对ZFTA融合室管膜瘤这一新定义的幕上肿瘤的认识和诊疗提供参考价值。

Ependymomas encompass a diverse array of subtypes,occurring in the supratentorial space,posterioRfossa,oRspinal cord.The World Health Organization(WHO)has periodically revised the classification system foRependymal tumors to account for specific anatomical features,histology,and genetic phenotypes.These updates have been instrumental in predicting and evaluating treatment outcomes foRpatients.In 2021,WHO furtheRupdated the classification to include ZFTA fusion-positive ependymoma as a distinct subtype due to its involvement of gene fusions such as RELA,MAML2/3,NCOA1/2,MN1 oRCTNNA2.This papeRreviewed the evolving classification criteria and treatment advancements foRsupratentorial ependymoma while summarizing research progress on clinical manifestations and treatment strategies foRZFTA fused ependymoma in ordeRto provide valuable insights into understanding,diagnosing and treating this newly defined supratentorial tumor.