Evidence map›Paper›PMID 38717379›Full record

ArticleNeuro-oncology2024

Gliomatosis cerebri in children: A poor prognostic phenotype of diffuse gliomas with a distinct molecular profile.

Gunther Nussbaumer, Martin Benesch, Yura Grabovska, Alan Mackay, David Castel, Jacques Grill, Marta M Alonso, Manila Antonelli, Simon Bailey, Joshua N Baugh and 65 more

Abstract readMulticenter Study
PubMed Full text at PMC Publisher
In one paragraph

Article in Neuro-oncology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.

0numbers the graph read from it
0cells of the map it votes in
11citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

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

11 citing papers in PubMed.

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4 · The record

Corrections and comments

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

75 authors.

Gunther NussbaumerDivision of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria.ORCID 0000-0003-0397-935X
Martin BeneschDivision of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria.
Yura GrabovskaDivision of Molecular Pathology, Institute of Cancer Research, London, UK.
Alan MackayDivision of Molecular Pathology, Institute of Cancer Research, London, UK.
David CastelU981, Molecular Predictors and New Targets in Oncology, Team Genomics and Oncogenesis of Pediatric Brain Tumors, INSERM, Gustave Roussy, Université Paris-Saclay, Villejuif, France.
Jacques GrillU981, Molecular Predictors and New Targets in Oncology, Team Genomics and Oncogenesis of Pediatric Brain Tumors, INSERM, Gustave Roussy, Université Paris-Saclay, Villejuif, France.
Marta M AlonsoDepartment of Pediatrics, Clínica Universidad de Navarra, Pamplona, Spain.
Manila AntonelliDepartment of Radiological, Oncological and Anatomo-Pathological Sciences, Sapienza University, Rome, Italy.
Simon BaileyDepartment of Paediatric Oncology, Sir James Spence Institute of Child Health, Royal Victoria Infirmary Queen Victoria Road, Newcastle upon Tyne, UK.
Joshua N BaughPrincess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.ORCID 0000-0001-7281-6306
Veronica BiassoniPediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
Mirjam Blattner-JohnsonDivision of Pediatric Glioma Research, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK), Heidelberg, Germany.
Alberto BroniscerDivision of Hematology-Oncology, Children's Hospital of Pittsburgh, Pittsburgh, PA, U.S.A.
Andrea CaraiNeurosurgery Unit, Bambino Gesù Children's Hospital (IRCCS), Rome, Italy.ORCID 0000-0002-5203-7855
Giovanna Stefania ColafatiOncological Neuroradiology and Advanced Diagnostics Unit, Bambino Gesù Children's Hospital (IRCCS), Rome, Italy.
Niclas ColditzDivision of Pediatric Hematology and Oncology, University Medical Center Göttingen, Göttingen, Germany.
Selim CorbaciogluDepartment of Pediatric Hematology, Oncology and Stem Cell Transplantation, University of Regensburg, Regensburg, Germany.
Shauna CrampsieDivision of Molecular Pathology, Institute of Cancer Research, London, UK.
Natacha Entz-WerleUMR CNRS 7021-Laboratory of Bioimagery and Pathologies Team tumor signaling and therapeutic targets, University of Strasbourg, Illkirch, France.
Matthias EyrichPediatric Hematology and Oncology, Department of Pediatrics, University Hospital Würzburg, Würzburg, Germany.
Lea L FrikerInstitute of Neuropathology, DGNN Brain Tumor Reference Center, University of Bonn Medical Center, Bonn, Germany.
Michael C FrühwaldPediatric and Adolescent Medicine, Swabian Children's Cancer Center, University Medical Center Augsburg, Augsburg, Germany.
Maria Luisa GarrèNeuro-Oncology Unit, IRCSS Istituto Giannina Gaslini, Genoa, Italy.
Nicolas U GerberDepartment of Oncology and Children's Research Centre, University Children's Hospital, Zurich, Switzerland.
Felice GiangasperoDepartment of Radiological, Oncological and Anatomo-Pathological Sciences, Sapienza University, Rome, Italy.
Maria J Gil-da-CostaPediatric Oncology Department, University Hospital São João, Porto, Portugal.
Norbert GrafDepartment of Pediatric Oncology and Hematology, Saarland University, Homburg, Germany.
Darren HargravePediatric Oncology, Great Ormond Street Hospital for Children, London, UK.
Peter HauserSecond Department of Pediatrics, Semmelweis University, Budapest, Hungary.
Ulrich HerrlingerDivision of Clinical Neuro-Oncology, Department of Neurology, University Hospital Bonn, Bonn, Germany.
Marion HoffmannDivision of Pediatric Hematology and Oncology, University Medical Center Göttingen, Göttingen, Germany.
Esther HullemanPrincess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Elisa IzquierdoDivision of Molecular Pathology, Institute of Cancer Research, London, UK.
Sandra JacobsDepartment of Pediatric Hematology and Oncology, University Hospitals Leuven, Belgium.
Michael KarremannDepartment of Pediatric and Adolescent Medicine, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany.
Antonis KattamisDivision of Pediatric Hematology-Oncology, First Department of Pediatrics, National and Kapodistrian University of Athens, "Aghia Sophia" Children's Hospital, Athens, Greece.
Rejin KebudiDivision of Pediatric Hematology-Oncology, Institute of Oncology, Istanbul University, Istanbul, Turkey.
Rolf-Dieter KortmannDepartment of Radiation Oncology, University of Leipzig, Leipzig, Germany.ORCID 0000-0002-8952-1819
Robert KwiecienInstitute of Biostatistics and Clinical Research, University of Münster, Münster, Germany.
Maura MassiminoPediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
Angela MastronuzziDepartment of Onco-Hematology, Gene and Cell Therapy, Bambino Gesù Children's Hospital (IRCCS), Rome, Italy.ORCID 0000-0002-4408-2373
Evelina MieleDepartment of Onco-Hematology, Gene and Cell Therapy, Bambino Gesù Children's Hospital (IRCCS), Rome, Italy.
Giovanni MoranaDepartment of Neurosciences, University of Turin, Turin, Italy.ORCID 0000-0001-8707-5969
Claudia M NoackCenter of Radiology, Department of Diagnostic and Interventional Radiology and Neuroradiology, Hospital of Fulda, Fulda, Germany.
Virve PentikainenDivision of Hematology-Oncology and Stem Cell Transplantation, Children's Hospital, Helsinki University Hospital, Helsinki, Finland.
Thomas PerweinDivision of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria.ORCID 0000-0001-6952-1339
Stefan M PfisterNational Center for Tumor Diseases (NCT) Heidelberg, Heidelberg, Germany.
Torsten PietschInstitute of Neuropathology, DGNN Brain Tumor Reference Center, University of Bonn Medical Center, Bonn, Germany.
Kleoniki RokaDivision of Pediatric Hematology-Oncology, First Department of Pediatrics, National and Kapodistrian University of Athens, "Aghia Sophia" Children's Hospital, Athens, Greece.
Sabrina RossiPathology Unit, Bambino Gesù Children's Hospital (IRCCS), Rome, Italy.
Stefan RutkowskiDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Elisabetta SchiavelloPediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
Clemens SeidelDepartment of Radiation Oncology, University of Leipzig, Leipzig, Germany.
Jaroslav ŠtěrbaDepartment of Pediatric Oncology, University Hospital Brno and Faculty of Medicine, Masaryk University, Brno, Czech Republic.
Dominik SturmDepartment of Pediatric Oncology, Hematology & Immunology, Heidelberg University Hospital, Heidelberg, Germany.
David SumerauerDepartment of Pediatric Hematology and Oncology, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.
Anna TackeDivision of Pediatric Hematology and Oncology, University Medical Center Göttingen, Göttingen, Germany.
Sara TemelsoDivision of Molecular Pathology, Institute of Cancer Research, London, UK.
Chiara ValentiniDepartment of Radiotherapy and Radiation Oncology, Faculty of Medicine and University Hospital Carl Gustav Carus, Technical University Dresden, Dresden, Germany.
Dannis van VuurdenPrincess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Pascale VarletGHU-Paris Psychiatry and Neuroscience, Sainte-Anne Hospital, Department of Neuropathology, Paris, France.
Sophie E M Veldhuijzen van ZantenDepartment of Radiology and Nuclear Medicine, Erasmus MC, Rotterdam, The Netherlands.
Maria VinciDepartment of Onco-Hematology, Gene and Cell Therapy, Bambino Gesù Children's Hospital (IRCCS), Rome, Italy.ORCID 0000-0002-7952-6771
André O von BuerenDepartment of Pediatrics, Obstetrics and Gynecology, Division of Pediatric Hematology and Oncology, University Hospital Geneva, Geneva, Switzerland.
Monika Warmuth-MetzInstitute of Diagnostic and Interventional Neuroradiology, University of Würzburg, Würzburg, Germany.
Pieter WesselingDepartment of Pathology, Amsterdam UMC, Amsterdam, The Netherlands.
Maria WieseNeurosurgery Unit, Bambino Gesù Children's Hospital (IRCCS), Rome, Italy.
Johannes E A WolffAbbVie Inc, Oncology Development, North Chicago, IL, U.S.A.
Josef ZamecnikDepartment of Pathology and Molecular Medicine, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.
Andrés Morales La MadridPediatric Neuro-Oncology, Pediatric Cancer Center Barcelona, Hospital Sant Joan de Deu, Barcelona, Spain.
Brigitte BisonNeuroradiological Reference Center for the Pediatric Brain Tumor (HIT) Studies of the German Society of Pediatric Oncology and Hematology, University Augsburg, Faculty of Medicine, Germany.
Gerrit H GielenInstitute of Neuropathology, DGNN Brain Tumor Reference Center, University of Bonn Medical Center, Bonn, Germany.
David T W JonesDivision of Pediatric Glioma Research, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK), Heidelberg, Germany.
Chris JonesDivision of Molecular Pathology, Institute of Cancer Research, London, UK.
Christof M KrammDivision of Pediatric Hematology and Oncology, University Medical Center Göttingen, Göttingen, Germany.

Funding

Cancer Research UK C13468/A23536Charles University Grant AgencyCRIS Cancer FoundationEtoile de MartinFondazione HealGerman- and Styrian Childhood Cancer FoundationIl Coraggio dei BambiniIl laboratorio di ChiaraItalian Ministry of HealthIzas, la Princesa Guisante Foundation PRIMUS/19/MED/06Joshua Bembo Project and the AYJ FundLes Boucles du Cœur de la Fondation CarrefourMartina e La Sua LunaNecker Imagine Tumor and DNA biobank BB-033-00065NHSNIHR Great Ormond Street Hospital Biomedical Research CentreOllie Young FoundationRegione Liguria NET-2019-12371188Regione Lombardia NET-2019-12371188Rudy A Menon Foundation
6 · The paper itself

Abstract

backgroundThe term gliomatosis cerebri (GC), a radiology-defined highly infiltrating diffuse glioma, has been abandoned since molecular GC-associated features could not be established.

methodsWe conducted a multinational retrospective study of 104 children and adolescents with GC providing comprehensive clinical and (epi-)genetic characterization.

resultsMedian overall survival (OS) was 15.5 months (interquartile range, 10.9-27.7) with a 2-year survival rate of 28%. Histopathological grading correlated significantly with median OS: CNS WHO grade II: 47.8 months (25.2-55.7); grade III: 15.9 months (11.4-26.3); grade IV: 10.4 months (8.8-14.4). By DNA methylation profiling (n = 49), most tumors were classified as pediatric-type diffuse high-grade glioma (pedHGG), H3-/IDH-wild-type (n = 31/49, 63.3%) with enriched subclasses pedHGG_RTK2 (n = 19), pedHGG_A/B (n = 6), and pedHGG_MYCN (n = 5), but only one pedHGG_RTK1 case. Within the pedHGG, H3-/IDH-wild-type subgroup, recurrent alterations in EGFR (n = 10) and BCOR (n = 9) were identified. Additionally, we observed structural aberrations in chromosome 6 in 16/49 tumors (32.7%) across tumor types. In the pedHGG, H3-/IDH-wild-type subgroup TP53 alterations had a significant negative effect on OS.

conclusionsContrary to previous studies, our representative pediatric GC study provides evidence that GC has a strong predilection to arise on the background of specific molecular features (especially pedHGG_RTK2, pedHGG_A/B, EGFR and BCOR mutations, chromosome 6 rearrangements).

Indexed as

Brain NeoplasmsGliomaNeoplasms, NeuroepithelialAdolescentBiomarkers, TumorChildChild, PreschoolDNA MethylationFemaleFollow-Up StudiesHumansInfantMaleMutationNeoplasm GradingPhenotypeBiomarkers, Tumorchromosome 6gliomatosis cerebriH3-wild-type and IDH-wild-typepedHGG_RTK2pediatric-type gliomapediatric-type high-grade glioma

Identifiers

PMID38717379
PMCPMC11376460

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.