室管膜瘤（EPN）不是一种统一的疾病，而是代表具有生物学和临床异质性的不同疾病类型。然而，不同类型 EPN 复发的时间和地点尚未得到全面描述。我们收集了来自欧洲和北美队列的 269 例复发性颅内 EPN 患者（n=233）和成人（n=36），并进行了相关 DNA 甲基化分析该队列包括以下分子 EPN 类型：PF-EPN-A (n=177)、ST-EPN-ZFTA (n=45)、PF-EPN-B (n=31) )、PF-EPN-SE (n=12) 和 ST-EPN-YAP (n=4)。 PF-EPN-B（PF：后颅窝）和 PF-EPN-SE（SE：室管膜下瘤）的首次复发晚于 PF-EPN-A、ST-EPN-YAP（ST：幕上）或 ST- EPN-ZFTA（中位复发时间：4.3 和 6.0 年对比 1.9/1.0/2.4 年；p<0.01）。与 ST-EPN-ZFTA 相比，PF-EPN-B 和 -A 中的转移性或联合复发更常累及脊髓（72.7% 和 40.0 vs. 12.5%；p<0.01）。 ST-EPN-YAP (n=4) 或 PF-EPN-SE (n=12) 中未观察到远处复发。 PF-EPN-A 和 ST-EPN-ZFTA 的复发后生存率 (PRS) 较差（5 年 PRS：44.5±4.4/47.8±9.1%），而 PF-EPN-B 和 PF-EPN-SE 显示5 年 PRS 为 89.5±7.1/90.0±9.5% (p=0.03)。然而，PF-EPN-B 的 10 年 PRS 下降至 45.8±17.3%。放射野与复发模式之间以及放射野与复发时脊柱受累之间均未发现影响。值得注意的是，所有复发 ST-EPN-YAP 患者均未接受前期放疗，但在复发时通过放疗成功挽救了病情。特定 EPN 类型的复发模式不同。未来的临床试验、治疗调整、监测和诊断持续时间应结合实体特定的复发信息进行规划。© 作者 2024。由牛津大学出版社代表神经肿瘤学会出版。版权所有。如需商业重复使用，请联系 reprints@oup.com 获取转载和转载的翻译权。所有其他权限均可通过我们网站文章页面上的“权限”链接通过我们的 RightsLink 服务获得 - 欲了解更多信息，请联系journals.permissions@oup.com。

Ependymoma (EPN) is not a uniform disease but represents different disease types with biological and clinical heterogeneity. However, the pattern of when and where different types of EPN relapse is not yet comprehensively described.We assembled 269 relapsed intracranial EPN from pediatric (n=233) and adult (n=36) patients from European and Northern American cohorts and correlated DNA methylation patterns and copy-number alterations with clinical information.The cohort comprised the following molecular EPN types: PF-EPN-A (n=177), ST-EPN-ZFTA (n=45), PF-EPN-B (n=31), PF-EPN-SE (n=12), and ST-EPN-YAP (n=4). First relapses of PF-EPN-B (PF: posterior-fossa) and PF-EPN-SE (SE: subependymoma) occurred later than of PF-EPN-A, ST-EPN-YAP (ST: supratentorial), or ST-EPN-ZFTA (median time to relapse: 4.3 and 6.0 years vs. 1.9/1.0/2.4 years; p<0.01). Metastatic or combined recurrences in PF-EPN-B and -A more often involved the spinal cord than in ST-EPN-ZFTA (72.7% and 40.0 vs. 12.5%; p<0.01). No distant relapses were observed in ST-EPN-YAP (n=4) or PF-EPN-SE (n=12). Post-relapse survival (PRS) was poor for PF-EPN-A and ST-EPN-ZFTA (5-year PRS: 44.5±4.4/47.8±9.1%), whereas PF-EPN-B and PF-EPN-SE displayed a 5-year PRS of 89.5±7.1/90.0±9.5% (p=0.03). However, 10-year PRS for PF-EPN-B dropped to 45.8±17.3%. Neither between radiation field and relapse pattern nor between radiation field and spinal involvement at relapse an impact was identified. Notably, all patients with relapsed ST-EPN-YAP did not receive upfront radiotherapy, but were successfully salvaged using irradiation at relapse.Relapse patterns of specific EPN types are different. Future clinical trials, treatment adaptions, duration of surveillance and diagnostics should be planned incorporating entity-specific relapse information.© The Author(s) 2024. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.