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000305024 1001_ $$avon Hoff, Katja$$b0
000305024 245__ $$aDeveloping an advanced risk stratification model for pediatric intracranial ependymoma based on the prospective trial E-HIT2000 and subsequent registries.
000305024 260__ $$aOxford$$bOxford Univ. Press$$c2025
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000305024 520__ $$aCurrent treatment strategies for pediatric intracranial ependymoma do not consider molecular heterogeneity. Here, we evaluated molecular group-specific determinants of outcome and developed an improved risk stratification model.Patients aged 0-21 years with localized intracranial ependymoma were enrolled into the prospective clinical trial E-HIT2000. Treatment included maximum safe surgery, local radiotherapy, and chemotherapy, stratified according to age, histology and, following a major amendment, residual tumor. Clinical data were analyzed in a pooled molecularly annotated cohort with data from patients treated analogously within subsequent registries.For 291 trial patients, the 5-year PFS and OS were 62±3% and 81±2%, respectively. For the molecularly annotated pooled cohort (n = 228), 5-year PFS/OS were: EPN-PFA (n = 146): 45±4%/77±4%; EPN-PFB (n = 19): 90±7%/100%; EPN-ZFTA (n = 59): 64±7%/86±5%; EPN-YAP1 (n = 4): 50±25%/100%. Patients with EPN-PFA without molecular risk factors (1q gain, and/or subtype EPN-PFA1c/d/e,2a), with complete resection, and postoperative radiotherapy showed favorable outcomes (5-year PFS/OS 75±10%/92±7%). For patients with EPN-PFA with molecular risk factors, prognosis was poor irrespective of residual tumor status (5-year PFS/OS: 33±6%/64±6%). Among EPN-ZFTA, 11/59 tumors were classified as EPN-ZFTA with alternative fusions, associated with inferior PFS (5-year PFS/OS: 36±15%/91±9%). For EPN-ZFTA-RELA, homozygous deletions of CDKN2A were associated with unfavorable outcomes (4-year PFS/OS: 19±16%/57±18% vs. 79±7%/97±3%, p = 0.0001). Finally, we developed a novel stratification model that discriminates standard and intermediate risk patients from those at high risk (p < 0.0001 for PFS and OS).These results strongly suggest the inclusion of molecular parameters into stratification, and the use of distinct treatment strategies within future ependymoma trials.
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000305024 650_7 $$2Other$$aClinical trial
000305024 650_7 $$2Other$$aDNA methylation profiling
000305024 650_7 $$2Other$$aEpendymoma
000305024 650_7 $$2Other$$aMolecular stratification
000305024 650_7 $$2Other$$aRisk stratification
000305024 7001_ $$aObrecht-Sturm, Denise$$b1
000305024 7001_ $$0P:(DE-HGF)0$$aGhasemi, David R$$b2$$eFirst author
000305024 7001_ $$aWenning, Janna$$b3
000305024 7001_ $$aMynarek, Martin$$b4
000305024 7001_ $$aGerber, Nicolas U$$b5
000305024 7001_ $$aBenesch, Martin$$b6
000305024 7001_ $$aJuhnke, Björn O$$b7
000305024 7001_ $$aBison, Brigitte$$b8
000305024 7001_ $$aWarmuth-Metz, Monika$$b9
000305024 7001_ $$aTimmermann, Beate$$b10
000305024 7001_ $$aFaldum, Andreas$$b11
000305024 7001_ $$aTippelt, Stephan$$b12
000305024 7001_ $$aFleischhack, Gudrun$$b13
000305024 7001_ $$aGrotzer, Michael$$b14
000305024 7001_ $$aDriever, Pablo Hernáiz$$b15
000305024 7001_ $$aBeilken, Andreas$$b16
000305024 7001_ $$aEbinger, Martin$$b17
000305024 7001_ $$aGraf, Norbert$$b18
000305024 7001_ $$aFrühwald, Michael C$$b19
000305024 7001_ $$aSchmid, Irene$$b20
000305024 7001_ $$aSlavc, Irene$$b21
000305024 7001_ $$aKoch, Arend$$b22
000305024 7001_ $$aBergmann, Markus$$b23
000305024 7001_ $$aHagel, Christian$$b24
000305024 7001_ $$aCoras, Roland$$b25
000305024 7001_ $$aBlümcke, Ingmar$$b26
000305024 7001_ $$0P:(DE-HGF)0$$aReifenberger, Guido$$b27
000305024 7001_ $$aFelsberg, Jörg$$b28
000305024 7001_ $$aKeyvani, Kathy$$b29
000305024 7001_ $$0P:(DE-He78)b15b56a6ed37417d476470c60c0140ff$$aHarter, Patrick$$b30
000305024 7001_ $$aPrinz, Marco$$b31
000305024 7001_ $$aStaszewski, Ori$$b32
000305024 7001_ $$aAcker, Till$$b33
000305024 7001_ $$aStadelmann-Nessler, Christine$$b34
000305024 7001_ $$aHartmann, Christian$$b35
000305024 7001_ $$0P:(DE-He78)a8a10626a848d31e70cfd96a133cc144$$avon Deimling, Andreas$$b36$$udkfz
000305024 7001_ $$aSommer, Clemens$$b37
000305024 7001_ $$aHasselblatt, Martin$$b38
000305024 7001_ $$aRiemenschneider, Markus J$$b39
000305024 7001_ $$aMonoranu, Camelia-Maria$$b40
000305024 7001_ $$aRushing, Elisabeth$$b41
000305024 7001_ $$aHaberler, Christine$$b42
000305024 7001_ $$0P:(DE-He78)4c28e2aade5f44d8eca9dd8e97638ec8$$aKool, Marcel$$b43$$udkfz
000305024 7001_ $$0P:(DE-He78)45440b44791309bd4b7dbb4f73333f9b$$aSill, Martin$$b44$$udkfz
000305024 7001_ $$0P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aPfister, Stefan M$$b45$$udkfz
000305024 7001_ $$aSchüller, Ulrich$$b46
000305024 7001_ $$aPietsch, Torsten$$b47
000305024 7001_ $$aKortmann, Rolf D$$b48
000305024 7001_ $$aKwiecien, Robert$$b49
000305024 7001_ $$0P:(DE-He78)046fd145f1008f83f6236580727bbc0f$$aWitt, Hendrik$$b50
000305024 7001_ $$0P:(DE-He78)a7c1bbac024fa232d9c6b78443328d9d$$aPajtler, Kristian W$$b51$$eLast author$$udkfz
000305024 7001_ $$aRutkowski, Stefan$$b52
000305024 7001_ $$aGerman, Austrian$$b53
000305024 7001_ $$aHIT-Network, Swiss$$b54$$eCollaboration Author
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