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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$$c2025
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000292426 500__ $$a Volume 27, Issue 1, January 2025, Pages 267–276
000292426 520__ $$aEpendymoma (EPN) is not a uniform disease but represents different disease types with biological and clinical heterogeneity. However, the pattern of when and where different types of EPN relapse is not yet comprehensively described.We assembled 269 relapsed intracranial EPN from pediatric (n=233) and adult (n=36) patients from European and Northern American cohorts and correlated DNA methylation patterns and copy-number alterations with clinical information.The cohort comprised the following molecular EPN types: PF-EPN-A (n=177), ST-EPN-ZFTA (n=45), PF-EPN-B (n=31), PF-EPN-SE (n=12), and ST-EPN-YAP (n=4). First relapses of PF-EPN-B (PF: posterior-fossa) and PF-EPN-SE (SE: subependymoma) occurred later than of PF-EPN-A, ST-EPN-YAP (ST: supratentorial), or ST-EPN-ZFTA (median time to relapse: 4.3 and 6.0 years vs. 1.9/1.0/2.4 years; p<0.01). Metastatic or combined recurrences in PF-EPN-B and -A more often involved the spinal cord than in ST-EPN-ZFTA (72.7% and 40.0 vs. 12.5%; p<0.01). No distant relapses were observed in ST-EPN-YAP (n=4) or PF-EPN-SE (n=12). Post-relapse survival (PRS) was poor for PF-EPN-A and ST-EPN-ZFTA (5-year PRS: 44.5±4.4/47.8±9.1%), whereas PF-EPN-B and PF-EPN-SE displayed a 5-year PRS of 89.5±7.1/90.0±9.5% (p=0.03). However, 10-year PRS for PF-EPN-B dropped to 45.8±17.3%. Neither between radiation field and relapse pattern nor between radiation field and spinal involvement at relapse an impact was identified. Notably, all patients with relapsed ST-EPN-YAP did not receive upfront radiotherapy, but were successfully salvaged using irradiation at relapse.Relapse patterns of specific EPN types are different. Future clinical trials, treatment adaptions, duration of surveillance and diagnostics should be planned incorporating entity-specific relapse information.
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000292426 650_7 $$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
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