000292426 001__ 292426 000292426 005__ 20250113142142.0 000292426 0247_ $$2doi$$a10.1093/neuonc/noae166 000292426 0247_ $$2pmid$$apmid:39171767 000292426 0247_ $$2ISSN$$a1522-8517 000292426 0247_ $$2ISSN$$a1523-5866 000292426 0247_ $$2altmetric$$aaltmetric:166579901 000292426 037__ $$aDKFZ-2024-01709 000292426 041__ $$aEnglish 000292426 082__ $$a610 000292426 1001_ $$00000-0002-3216-8452$$aObrecht-Sturm, Denise$$b0 000292426 245__ $$aDistinct relapse pattern across molecular ependymoma types. 000292426 260__ $$aOxford$$bOxford Univ. Press$$c2025 000292426 3367_ $$2DRIVER$$aarticle 000292426 3367_ $$2DataCite$$aOutput Types/Journal article 000292426 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1736774457_12089 000292426 3367_ $$2BibTeX$$aARTICLE 000292426 3367_ $$2ORCID$$aJOURNAL_ARTICLE 000292426 3367_ $$00$$2EndNote$$aJournal Article 000292426 500__ $$a Volume 27, Issue 1, January 2025, Pages 267–276 000292426 520__ $$aEpendymoma (EPN) is not a uniform disease but represents different disease types with biological and clinical heterogeneity. However, the pattern of when and where different types of EPN relapse is not yet comprehensively described.We assembled 269 relapsed intracranial EPN from pediatric (n=233) and adult (n=36) patients from European and Northern American cohorts and correlated DNA methylation patterns and copy-number alterations with clinical information.The cohort comprised the following molecular EPN types: PF-EPN-A (n=177), ST-EPN-ZFTA (n=45), PF-EPN-B (n=31), PF-EPN-SE (n=12), and ST-EPN-YAP (n=4). First relapses of PF-EPN-B (PF: posterior-fossa) and PF-EPN-SE (SE: subependymoma) occurred later than of PF-EPN-A, ST-EPN-YAP (ST: supratentorial), or ST-EPN-ZFTA (median time to relapse: 4.3 and 6.0 years vs. 1.9/1.0/2.4 years; p<0.01). Metastatic or combined recurrences in PF-EPN-B and -A more often involved the spinal cord than in ST-EPN-ZFTA (72.7% and 40.0 vs. 12.5%; p<0.01). No distant relapses were observed in ST-EPN-YAP (n=4) or PF-EPN-SE (n=12). Post-relapse survival (PRS) was poor for PF-EPN-A and ST-EPN-ZFTA (5-year PRS: 44.5±4.4/47.8±9.1%), whereas PF-EPN-B and PF-EPN-SE displayed a 5-year PRS of 89.5±7.1/90.0±9.5% (p=0.03). However, 10-year PRS for PF-EPN-B dropped to 45.8±17.3%. Neither between radiation field and relapse pattern nor between radiation field and spinal involvement at relapse an impact was identified. Notably, all patients with relapsed ST-EPN-YAP did not receive upfront radiotherapy, but were successfully salvaged using irradiation at relapse.Relapse patterns of specific EPN types are different. Future clinical trials, treatment adaptions, duration of surveillance and diagnostics should be planned incorporating entity-specific relapse information. 000292426 536__ $$0G:(DE-HGF)POF4-312$$a312 - Funktionelle und strukturelle Genomforschung (POF4-312)$$cPOF4-312$$fPOF IV$$x0 000292426 588__ $$aDataset connected to CrossRef, PubMed, , Journals: inrepo02.dkfz.de 000292426 650_7 $$2Other$$aependymoma 000292426 650_7 $$2Other$$ametastatic 000292426 650_7 $$2Other$$arecurrence 000292426 650_7 $$2Other$$arelapse 000292426 7001_ $$aSchoof, Melanie$$b1 000292426 7001_ $$aEckhardt, Alicia$$b2 000292426 7001_ $$00000-0003-3302-2719$$aMynarek, Martin$$b3 000292426 7001_ $$00000-0003-2556-9722$$aGilbert, Mark R$$b4 000292426 7001_ $$aAldape, Kenneth$$b5 000292426 7001_ $$aArmstrong, Terri S$$b6 000292426 7001_ $$00000-0002-6557-895X$$aRamaswamy, Vijay$$b7 000292426 7001_ $$aBockmayr, Michael$$b8 000292426 7001_ $$avon Hoff, Katja$$b9 000292426 7001_ $$aFleischhack, Gudrun$$b10 000292426 7001_ $$aAdolph, Jonas E$$b11 000292426 7001_ $$aTippelt, Stephan$$b12 000292426 7001_ $$0P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aPfister, Stefan$$b13$$udkfz 000292426 7001_ $$0P:(DE-He78)a7c1bbac024fa232d9c6b78443328d9d$$aPajtler, Kristian$$b14$$udkfz 000292426 7001_ $$0P:(DE-He78)a46a5b2a871859c8e2d63d2f8c666807$$aSturm, Dominik$$b15$$udkfz 000292426 7001_ $$00000-0002-4830-3671$$aDrexler, Richard$$b16 000292426 7001_ $$aRicklefs, Franz L$$b17 000292426 7001_ $$aStepien, Natalia$$b18 000292426 7001_ $$aGojo, Johannes$$b19 000292426 7001_ $$aPietsch, Torsten$$b20 000292426 7001_ $$00000-0002-3544-319X$$aWarmuth-Metz, Monika$$b21 000292426 7001_ $$aKortmann, Rolf$$b22 000292426 7001_ $$aTimmermann, Beate$$b23 000292426 7001_ $$00000-0003-1016-0545$$aHaberler, Christine$$b24 000292426 7001_ $$aRutkowski, Stefan$$b25 000292426 7001_ $$00000-0002-8731-1121$$aSchüller, Ulrich$$b26 000292426 773__ $$0PERI:(DE-600)2094060-9$$a10.1093/neuonc/noae166$$gp. noae166$$n1$$p267–276$$tNeuro-Oncology$$v27$$x1522-8517$$y2025 000292426 909CO $$ooai:inrepo02.dkfz.de:292426$$pVDB 000292426 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aDeutsches Krebsforschungszentrum$$b13$$kDKFZ 000292426 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)a7c1bbac024fa232d9c6b78443328d9d$$aDeutsches Krebsforschungszentrum$$b14$$kDKFZ 000292426 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)a46a5b2a871859c8e2d63d2f8c666807$$aDeutsches Krebsforschungszentrum$$b15$$kDKFZ 000292426 9131_ $$0G:(DE-HGF)POF4-312$$1G:(DE-HGF)POF4-310$$2G:(DE-HGF)POF4-300$$3G:(DE-HGF)POF4$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lKrebsforschung$$vFunktionelle und strukturelle Genomforschung$$x0 000292426 9141_ $$y2024 000292426 915__ $$0StatID:(DE-HGF)0420$$2StatID$$aNationallizenz$$d2023-10-24$$wger 000292426 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bNEURO-ONCOLOGY : 2022$$d2023-10-24 000292426 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS$$d2023-10-24 000292426 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline$$d2023-10-24 000292426 915__ $$0StatID:(DE-HGF)0320$$2StatID$$aDBCoverage$$bPubMed Central$$d2023-10-24 000292426 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bClarivate Analytics Master Journal List$$d2023-10-24 000292426 915__ $$0StatID:(DE-HGF)0113$$2StatID$$aWoS$$bScience Citation Index Expanded$$d2023-10-24 000292426 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection$$d2023-10-24 000292426 915__ $$0StatID:(DE-HGF)0160$$2StatID$$aDBCoverage$$bEssential Science Indicators$$d2023-10-24 000292426 915__ $$0StatID:(DE-HGF)1110$$2StatID$$aDBCoverage$$bCurrent Contents - Clinical Medicine$$d2023-10-24 000292426 915__ $$0StatID:(DE-HGF)9915$$2StatID$$aIF >= 15$$bNEURO-ONCOLOGY : 2022$$d2023-10-24 000292426 9201_ $$0I:(DE-He78)B360-20160331$$kB360$$lPädiatrische Gliomforschung$$x0 000292426 9201_ $$0I:(DE-He78)HD01-20160331$$kHD01$$lDKTK HD zentral$$x1 000292426 980__ $$ajournal 000292426 980__ $$aVDB 000292426 980__ $$aI:(DE-He78)B360-20160331 000292426 980__ $$aI:(DE-He78)HD01-20160331 000292426 980__ $$aUNRESTRICTED