Evidence map›Paper›PMID 41026848›Full record

ArticleNeuro-oncology2026

Developing an advanced risk stratification model for pediatric intracranial ependymoma based on the prospective trial E-HIT2000 and subsequent registries.

Katja von Hoff, Denise Obrecht-Sturm, David R Ghasemi, Janna Wenning, Martin Mynarek, Nicolas U Gerber, Martin Benesch, Björn O Juhnke, Brigitte Bison, Monika Warmuth-Metz and 44 more

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Article in Neuro-oncology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.

0numbers the graph read from it
0cells of the map it votes in
6citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

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Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

6 citing papers in PubMed.

  1. Article
  2. Review
  3. Review
  4. Article
  5. ZFTA::YAP1-rearranged ependymoma: is still ZFTA or already YAP?Acta neuropathologica communications · 2026
    Article
  6. A brief history of ependymoma.Neuro-oncology · 2026
    Review
4 · The record

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PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

54 authors.

Katja von HoffDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany (K.v.H., D.O.-S., D.R.G., J.W., M.M., B.O.J., U.S., S.R.).
Denise Obrecht-SturmDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany (K.v.H., D.O.-S., D.R.G., J.W., M.M., B.O.J., U.S., S.R.).ORCID 0000-0002-3216-8452
David R GhasemiDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany (K.v.H., D.O.-S., D.R.G., J.W., M.M., B.O.J., U.S., S.R.).ORCID 0000-0002-3844-9933
Janna WenningDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany (K.v.H., D.O.-S., D.R.G., J.W., M.M., B.O.J., U.S., S.R.).
Martin MynarekDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany (K.v.H., D.O.-S., D.R.G., J.W., M.M., B.O.J., U.S., S.R.).ORCID 0000-0003-3302-2719
Nicolas U GerberDepartment of Pediatric Oncology, University Children's Hospital Zürich, Zürich, Switzerland.
Martin BeneschDivision of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria (M.B.).
Björn O JuhnkeDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany (K.v.H., D.O.-S., D.R.G., J.W., M.M., B.O.J., U.S., S.R.).
Brigitte BisonDiagnostic and interventional Neuroradiology, Faculty of Medicine, University of Augsburg, Augsburg, Germany.
Monika Warmuth-MetzInstitute of Diagnostic and Interventional Neuroradiology, University Hospital Wuerzburg, Würzburg, Germany.ORCID 0000-0002-3544-319X
Beate TimmermannWest German Proton Therapy Center Essen (WPE), University Hospital Essen, Essen, Germany.
Andreas FaldumInstitute of Biostatistics and Clinical Research, Westfälische Wilhelms-Universität Münster, Münster, Germany.
Stephan TippeltPediatric Hematology and Oncology, Pediatrics III, Center for Translational Neuro- and Behavioral Sciences (CTNBS), University Hospital of Essen, Essen, Germany.
Gudrun FleischhackPediatric Hematology and Oncology, Pediatrics III, Center for Translational Neuro- and Behavioral Sciences (CTNBS), University Hospital of Essen, Essen, Germany.
Michael GrotzerDepartment of Pediatric Oncology, University Children's Hospital Zürich, Zürich, Switzerland.
Pablo Hernáiz DrieverDepartment of Pediatric Oncology and Hematology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany (K.v.H., P.H.D.).ORCID 0000-0003-3135-3872
Andreas BeilkenDepartment of Pediatrics, Klinikum Braunschweig gGmbH, Braunschweig, Germany.
Martin EbingerDepartment of Hematology and Oncology, University Children's Hospital Tübingen, Tuebingen, Germany.
Norbert GrafDepartment of Pediatric Haematology and Oncology, Saarland University Hospital, Homburg, Germany.
Michael C FrühwaldPediatrics and Adolescent Medicine, Swabian Childrens' Cancer Center, University Medical Center Augsburg, Augsburg, Germany, (M.C.F.).
Irene SchmidDivision of Pediatric Hematology and Oncology, Dr. von Hauner Children's Hospital, University Hospital Munich, LMU, Munich, Germany.
Irene SlavcDepartment of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.ORCID 0000-0002-9419-6790
Arend KochDepartment of Neuropathology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Germany.ORCID 0000-0001-8589-4688
Markus BergmannInstitute of Neuropathology, Center for Pathology, Klinikum Bremen Mitte, Bremen, Germany.
Christian HagelInstitute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.ORCID 0000-0003-0518-0824
Roland CorasDepartment of Neuropathology, University Hospital Erlangen, Member of EpiCARE ERN, Erlangen, Germany.
Ingmar BlümckeDepartment of Neuropathology, University Hospital Erlangen, Member of EpiCARE ERN, Erlangen, Germany.ORCID 0000-0001-8676-0788
Guido ReifenbergerInstitute of Neuropathology, Heinrich Heine University, Medical Faculty, University Hospital Düsseldorf, Düsseldorf, Germany.ORCID 0000-0002-1419-9837
Jörg FelsbergInstitute of Neuropathology, Heinrich Heine University, Medical Faculty, University Hospital Düsseldorf, Düsseldorf, Germany.
Kathy KeyvaniInstitute of Neuropathology, University of Duisburg-Essen, Essen, Germany.
Patrick N HarterInstitute of Neurology (Edinger Institute), Goethe-University Frankfurt am Main, Frankfurt am Main, Germany.
Marco PrinzInstitute of Neuropathology, Medical Faculty, University of Freiburg, Freiburg, Germany.ORCID 0000-0002-0349-1955
Ori StaszewskiInstitute of Neuropathology, Medical Faculty, University of Freiburg, Freiburg, Germany.
Till AckerInstitute of Neuropathology, University of Giessen, Giessen, Germany.
Christine Stadelmann-NesslerInstitute of Neuropathology, University Medical Center, Göttingen, Germany.
Christian HartmannDepartment for Neuropathology, Institute for Pathology, Hannover Medical School, Hannover, Germany.
Andreas von DeimlingDepartment of Neuropathology, University Hospital Heidelberg, Heidelberg, Germany.ORCID 0000-0002-5863-540X
Clemens SommerDepartment of Neuropathology, University Medical Centre, Johannes Gutenberg-University of Mainz, Mainz, Germany.
Martin HasselblattInstitute of Neuropathology, University Hospital Münster, Münster, Germany.ORCID 0000-0003-2707-8484
Markus J RiemenschneiderDepartment of Neuropathology, Regensburg University Hospital, Regensburg, Germany.
Camelia-Maria MonoranuDepartment of Neuropathology, Institute for Pathology, University of Würzburg, Würzburg, Germany.
Elisabeth RushingDivision of Neuropathology, University Hospital Zurich, Zurich, Switzerland.
Christine HaberlerDepartment of Neurology, Division of Neuropathology and Neurochemistry, Medical University of Vienna, Vienna, Austria.ORCID 0000-0003-1016-0545
Marcel KoolHopp Children's Cancer Center Heidelberg (KiTZ), Heidelberg, Germany.
Martin SillHopp Children's Cancer Center Heidelberg (KiTZ), Heidelberg, Germany.ORCID 0000-0001-7616-7665
Stefan M PfisterHopp Children's Cancer Center Heidelberg (KiTZ), Heidelberg, Germany.ORCID 0000-0002-5447-5322
Ulrich SchüllerDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany (K.v.H., D.O.-S., D.R.G., J.W., M.M., B.O.J., U.S., S.R.).ORCID 0000-0002-8731-1121
Torsten PietschDepartment of Neuropathology, DGNN Brain Tumor Reference Center, University of Bonn Medical Center, Bonn, Germany.
Rolf D KortmannDepartment of Radiation Oncology, University of Leipzig, Leipzig, Germany.
Robert KwiecienInstitute of Biostatistics and Clinical Research, Westfälische Wilhelms-Universität Münster, Münster, Germany.
Hendrik WittHopp Children's Cancer Center Heidelberg (KiTZ), Heidelberg, Germany.
Kristian W PajtlerHopp Children's Cancer Center Heidelberg (KiTZ), Heidelberg, Germany.ORCID 0000-0002-3562-6121
Stefan RutkowskiDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany (K.v.H., D.O.-S., D.R.G., J.W., M.M., B.O.J., U.S., S.R.).ORCID 0000-0002-5446-9571
German, Austrian and Swiss HIT-Network

Funding

Deutsche Kinderkrebsstiftung 2009.19Deutsche Kinderkrebsstiftung 2011.01Deutsche Kinderkrebsstiftung 2014.17Deutsche Kinderkrebsstiftung DKS 2006.03German Academic Scholarship FoundationGerman Brain Tumor Reference CenterMildred Scheel Nachwuchszentrum HamburgStudienstiftung des Deutschen VolkesThe HIT-MED trial office DKS 2001.03The HIT-MED trial office DKS 2004.03The HIT-MED trial office DKS 2005.13
6 · The paper itself

Abstract

abstractBackgroundCurrent 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.

methodsPatients 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.

resultsFor 291 trial patients, the 5-year progression-free survival (PFS) and overall survival (OS) were 62 ± 3% and 81 ± 2%, respectively. For the molecularly annotated pooled cohort (n = 228), 5-year PFS/OS were: posterior-fossa group A ependymoma (EPN-PFA) (n = 146): 45 ± 4%/77 ± 4%; posterior-fossa group B ependymoma (EPN-PFB) (n = 19): 90 ± 7%/100%; supratentorial ependymoma, ZFTA fusion-positive (EPN-ZFTA) (n = 59): 64 ± 7%/86 ± 5%; supratentorial ependymoma, YAP1 fusion-positive (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 = .0001). Finally, we developed a novel stratification model that discriminates standard and intermediate risk patients from those at high risk (P < .0001 for PFS and OS).

conclusionsThese results strongly suggest the inclusion of molecular parameters into stratification and the use of distinct treatment strategies within future ependymoma trials.

Indexed as

Brain NeoplasmsEpendymomaRegistriesAdolescentChildChild, PreschoolCombined Modality TherapyFemaleFollow-Up StudiesHumansInfantInfant, NewbornMalePrognosisProspective StudiesRisk Assessmentclinical trialDNA methylation profilingependymomamolecular ­stratificationrisk stratification

Identifiers

PMID41026848
PMCPMC12979045

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