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| Home > Publications database > Developing an advanced risk stratification model for pediatric intracranial ependymoma based on the prospective trial E-HIT2000 and subsequent registries. > print |
| 001 | 305024 | ||
| 005 | 20251005023007.0 | ||
| 024 | 7 | _ | |a 10.1093/neuonc/noaf218 |2 doi |
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| 037 | _ | _ | |a DKFZ-2025-02006 |
| 041 | _ | _ | |a English |
| 082 | _ | _ | |a 610 |
| 100 | 1 | _ | |a von Hoff, Katja |b 0 |
| 245 | _ | _ | |a Developing an advanced risk stratification model for pediatric intracranial ependymoma based on the prospective trial E-HIT2000 and subsequent registries. |
| 260 | _ | _ | |a Oxford |c 2025 |b Oxford Univ. Press |
| 336 | 7 | _ | |a article |2 DRIVER |
| 336 | 7 | _ | |a Output Types/Journal article |2 DataCite |
| 336 | 7 | _ | |a Journal Article |b journal |m journal |0 PUB:(DE-HGF)16 |s 1759325834_7599 |2 PUB:(DE-HGF) |
| 336 | 7 | _ | |a ARTICLE |2 BibTeX |
| 336 | 7 | _ | |a JOURNAL_ARTICLE |2 ORCID |
| 336 | 7 | _ | |a Journal Article |0 0 |2 EndNote |
| 500 | _ | _ | |a epub /#EA:B062#LA:B062# |
| 520 | _ | _ | |a Current 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. |
| 536 | _ | _ | |a 312 - Funktionelle und strukturelle Genomforschung (POF4-312) |0 G:(DE-HGF)POF4-312 |c POF4-312 |f POF IV |x 0 |
| 588 | _ | _ | |a Dataset connected to CrossRef, PubMed, , Journals: inrepo02.dkfz.de |
| 650 | _ | 7 | |a Clinical trial |2 Other |
| 650 | _ | 7 | |a DNA methylation profiling |2 Other |
| 650 | _ | 7 | |a Ependymoma |2 Other |
| 650 | _ | 7 | |a Molecular stratification |2 Other |
| 650 | _ | 7 | |a Risk stratification |2 Other |
| 700 | 1 | _ | |a Obrecht-Sturm, Denise |b 1 |
| 700 | 1 | _ | |a Ghasemi, David R |0 P:(DE-HGF)0 |b 2 |e First author |
| 700 | 1 | _ | |a Wenning, Janna |b 3 |
| 700 | 1 | _ | |a Mynarek, Martin |b 4 |
| 700 | 1 | _ | |a Gerber, Nicolas U |b 5 |
| 700 | 1 | _ | |a Benesch, Martin |b 6 |
| 700 | 1 | _ | |a Juhnke, Björn O |b 7 |
| 700 | 1 | _ | |a Bison, Brigitte |b 8 |
| 700 | 1 | _ | |a Warmuth-Metz, Monika |b 9 |
| 700 | 1 | _ | |a Timmermann, Beate |b 10 |
| 700 | 1 | _ | |a Faldum, Andreas |b 11 |
| 700 | 1 | _ | |a Tippelt, Stephan |b 12 |
| 700 | 1 | _ | |a Fleischhack, Gudrun |b 13 |
| 700 | 1 | _ | |a Grotzer, Michael |b 14 |
| 700 | 1 | _ | |a Driever, Pablo Hernáiz |b 15 |
| 700 | 1 | _ | |a Beilken, Andreas |b 16 |
| 700 | 1 | _ | |a Ebinger, Martin |b 17 |
| 700 | 1 | _ | |a Graf, Norbert |b 18 |
| 700 | 1 | _ | |a Frühwald, Michael C |b 19 |
| 700 | 1 | _ | |a Schmid, Irene |b 20 |
| 700 | 1 | _ | |a Slavc, Irene |b 21 |
| 700 | 1 | _ | |a Koch, Arend |b 22 |
| 700 | 1 | _ | |a Bergmann, Markus |b 23 |
| 700 | 1 | _ | |a Hagel, Christian |b 24 |
| 700 | 1 | _ | |a Coras, Roland |b 25 |
| 700 | 1 | _ | |a Blümcke, Ingmar |b 26 |
| 700 | 1 | _ | |a Reifenberger, Guido |0 P:(DE-HGF)0 |b 27 |
| 700 | 1 | _ | |a Felsberg, Jörg |b 28 |
| 700 | 1 | _ | |a Keyvani, Kathy |b 29 |
| 700 | 1 | _ | |a Harter, Patrick |0 P:(DE-He78)b15b56a6ed37417d476470c60c0140ff |b 30 |
| 700 | 1 | _ | |a Prinz, Marco |b 31 |
| 700 | 1 | _ | |a Staszewski, Ori |b 32 |
| 700 | 1 | _ | |a Acker, Till |b 33 |
| 700 | 1 | _ | |a Stadelmann-Nessler, Christine |b 34 |
| 700 | 1 | _ | |a Hartmann, Christian |b 35 |
| 700 | 1 | _ | |a von Deimling, Andreas |0 P:(DE-He78)a8a10626a848d31e70cfd96a133cc144 |b 36 |u dkfz |
| 700 | 1 | _ | |a Sommer, Clemens |b 37 |
| 700 | 1 | _ | |a Hasselblatt, Martin |b 38 |
| 700 | 1 | _ | |a Riemenschneider, Markus J |b 39 |
| 700 | 1 | _ | |a Monoranu, Camelia-Maria |b 40 |
| 700 | 1 | _ | |a Rushing, Elisabeth |b 41 |
| 700 | 1 | _ | |a Haberler, Christine |b 42 |
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| 700 | 1 | _ | |a Schüller, Ulrich |b 46 |
| 700 | 1 | _ | |a Pietsch, Torsten |b 47 |
| 700 | 1 | _ | |a Kortmann, Rolf D |b 48 |
| 700 | 1 | _ | |a Kwiecien, Robert |b 49 |
| 700 | 1 | _ | |a Witt, Hendrik |0 P:(DE-He78)046fd145f1008f83f6236580727bbc0f |b 50 |
| 700 | 1 | _ | |a Pajtler, Kristian W |0 P:(DE-He78)a7c1bbac024fa232d9c6b78443328d9d |b 51 |e Last author |u dkfz |
| 700 | 1 | _ | |a Rutkowski, Stefan |b 52 |
| 700 | 1 | _ | |a German, Austrian |b 53 |
| 700 | 1 | _ | |a HIT-Network, Swiss |b 54 |e Collaboration Author |
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