一种基于甲基化的室管膜瘤分型方法最近得到广泛应用。然而，这种分型方法的诊断优势和治疗决策的影响仍不明确。本文回顾性评估了分子重新分类后，手术和放射治疗对成年颅内室管膜瘤结果的影响。 收集来自八个诊断机构的170名成年患者的诊断为颅内室管膜瘤的肿瘤，进行了DNA甲基化分析。分子分类、患者特征和治疗与无进展生存期（PFS）相关。 分类器指示73.5％的例子为室管膜肿瘤，10.6％不同的肿瘤实体和15.9％不可分类的肿瘤实体。最常见的分子类别是后颅窝室管膜瘤B组（EPN-PFB，32.9％），后颅窝椎管下室管膜瘤（PF-SE，25.9％）和颅骨上ZFTA融合阳性室管膜瘤（EPN-ZFTA，11.2％）。在中位随访60.0个月的情况下，EPN-PFB的5年和10年PFS率分别为64.5％和41.8％，PF-SE的5年和10年PFS率分别为67.4％和45.2％，EPN-ZFTA的5年和10年PFS率分别为60.3％和60.3％。在EPN-PFB中，而不是在其他分子类别中，多变量分析中完全切除（p = 0.009）和术后放射治疗（p = 0.007）与改善PFS显著相关。分子定义的室管膜瘤类别内，组织学肿瘤分级（WHO 2与3）不是预测预后的预示因素。 DNA甲基化分析提高了成年颅内室管膜瘤的诊断准确性和风险分层。PF-SE分子类在具有室管膜瘤组织学的成年肿瘤中意外地非常常见，并像EPN-PFB一样频繁复发，尽管被认为是良性的。完全切除和放射治疗可能代表EPN-PFB患者结果的关键因素。 ©2023作者（Oxford University Press代表神经肿瘤学会出版）。保留所有权利。有关权限，请发送电子邮件至：journals.permissions@oup.com。

A methylation-based classification of ependymoma has recently found broad application. However, the diagnostic advantage and implications for treatment decisions remain unclear. Here, we retrospectively evaluate the impact of surgery and radiotherapy on outcome after molecular reclassification of adult intracranial ependymomas.Tumors diagnosed as intracranial ependymomas from 170 adult patients collected from eight diagnostic institutions were subjected to DNA methylation profiling. Molecular classes, patient characteristics, and treatment were correlated with progression-free survival (PFS).The classifier indicated an ependymal tumor in 73.5%, a different tumor entity in 10.6% and non-classifiable tumors in 15.9% of cases, respectively. The most prevalent molecular classes were posterior fossa ependymoma group B (EPN-PFB, 32.9%), posterior fossa subependymoma (PF-SE, 25.9%), and supratentorial ZFTA fusion-positive ependymoma (EPN-ZFTA, 11.2%). With a median follow-up of 60.0 months, the 5- and 10-year-PFS rates were 64.5% and 41.8% for EPN-PFB, 67.4% and 45.2% for PF-SE and 60.3% and 60.3% for EPN-ZFTA. In EPN-PFB, but not in other molecular classes, gross total resection (p=0.009) and postoperative radiotherapy (p=0.007) were significantly associated with improved PFS in multivariable analysis. Histological tumor grading (WHO 2 vs. 3) was not a predictor of prognosis within molecularly defined ependymoma classes.DNA methylation profiling improves diagnostic accuracy and risk stratification in adult intracranial ependymoma. The molecular class of PF-SE is unexpectedly prevalent among adult tumors with ependymoma histology and relapsed as frequently as EPN-PFB, despite the supposed benign nature. Gross total resection and radiotherapy may represent key factors in determining the outcome of EPN-PFB patients.© The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.