Ependymomas encompass a diverse array of subtypes,occurring in the supratentorial space,posterior fossa,or spinal cord.The World Health Organization(WHO)has periodically revised the classification system for ependymal tumors to account for specific anatomical features,histology,and genetic phenotypes.These updates have been instrumental in predicting and evaluating treatment outcomes for patients.In 2021,WHO further updated 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 or CTNNA2.This paper reviewed the evolving classification criteria and treatment advancements for supratentorial ependymoma while summarizing research progress on clinical manifestations and treatment strategies for ZFTA fused ependymoma in order to provide valuable insights into understanding,diagnosing and treating this newly defined supratentorial tumor.
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