Evidence map›Paper›PMID 41206756›Full record

SynthesisNeuro-oncology2026

A systematic study of molecular diagnosis, treatment, and prognosis in infant-type hemispheric glioma: An individual patient data meta-analysis of 164 patients.

Lara Chavaz, Aditi Bagchi, Sandeep K Dhanda, Fabienne Toutain, Stefan M Pfister, Dominik Sturm, Torsten Pietsch, Gerrit H Gielen, Andreas Waha, Matthew Clarke and 37 more

Abstract readMeta-Analysis
In one paragraph

Synthesis in Neuro-oncology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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5 · Who and what money

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47 authors.

Lara ChavazDepartment of Pediatrics, Gynecology and Obstetrics, Division of Pediatric Hematology and Oncology, University Hospital of Geneva, Geneva (L.C., F.T., A.O.v.B.).
Aditi BagchiDepartment of Oncology, Division of Neuro-Oncology, St. Jude Children's Research Hospital, Memphis (A.B., S.K.D., A.G., G.W.R.).ORCID 0000-0001-5466-7685
Sandeep K DhandaDepartment of Oncology, Division of Neuro-Oncology, St. Jude Children's Research Hospital, Memphis (A.B., S.K.D., A.G., G.W.R.).
Fabienne ToutainDepartment of Pediatrics, Gynecology and Obstetrics, Division of Pediatric Hematology and Oncology, University Hospital of Geneva, Geneva (L.C., F.T., A.O.v.B.).
Stefan M PfisterDepartment of Pediatric Oncology, Hematology & Immunology, Heidelberg University Hospital, Heidelberg (S.M.P., D.S.).ORCID 0000-0002-5447-5322
Dominik SturmDepartment of Pediatric Oncology, Hematology & Immunology, Heidelberg University Hospital, Heidelberg (S.M.P., D.S.).ORCID 0000-0003-0250-1696
Torsten PietschInstitute of Neuropathology, DGNN Brain Tumor Reference Center, University of Bonn, Bonn (T.P., G.H.G., A.W.).
Gerrit H GielenInstitute of Neuropathology, DGNN Brain Tumor Reference Center, University of Bonn, Bonn (T.P., G.H.G., A.W.).
Andreas WahaInstitute of Neuropathology, DGNN Brain Tumor Reference Center, University of Bonn, Bonn (T.P., G.H.G., A.W.).
Matthew ClarkeDivision of Molecular Pathology, Institute of Cancer Research, London (M.C., A.M., C.J.).
Congyu LuDepartment of Computational Biology, St. Jude Children's Research Hospital, Memphis (C.L., X.Z.).
Michael KarremannDepartment of Pediatric and Adolescent Medicine, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim (M.K.).ORCID 0000-0002-9961-4752
Martin BeneschDivision of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz (M.B., T.P., G.N.).
Thomas PerweinDivision of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz (M.B., T.P., G.N.).ORCID 0000-0001-6952-1339
Gunther NussbaumerDivision of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz (M.B., T.P., G.N.).
Christof KrammDivision of Pediatric Hematology and Oncology, University Medical Center Göttingen, Göttingen (C.K.).
Maura MassiminoPediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Milan (M.M., V.B.).ORCID 0000-0002-5506-2001
Veronica BiassoniPediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Milan (M.M., V.B.).ORCID 0000-0003-1486-1736
Maria VinciResearch Area of Onco-Hematology and Pharmaceutical GMP Facility, Bambino Gesù Children's Hospital IRCCS, Rome (M.V., A.M.).ORCID 0000-0002-7952-6771
Angela MastronuzziResearch Area of Onco-Hematology and Pharmaceutical GMP Facility, Bambino Gesù Children's Hospital IRCCS, Rome (M.V., A.M.).ORCID 0000-0002-4408-2373
Dannis van VuurdenPrincess Máxima Center for Pediatric Oncology, Utrecht (D.v.V.).
Sophie E M Veldhuijzen van ZantenDepartment of Radiology and Nuclear Medicine, Erasmus MC, Rotterdam (S.E.M.V.v.Z.).
Alan MackayDivision of Molecular Pathology, Institute of Cancer Research, London (M.C., A.M., C.J.).
Chris JonesDivision of Molecular Pathology, Institute of Cancer Research, London (M.C., A.M., C.J.).
David T W JonesDivision of Pediatric Glioma Research, Hopp Children's Cancer Center Heidelberg (KiTZ), Heidelberg (D.S., D.T.W.J.).
Ana S Guerreiro StucklinDepartment of Oncology and Children's Research Center, University Children's Hospital Zurich, Zurich (A.S.G.S.).
Uri TaboriThe Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto (U.T., C.H., S.R.).ORCID 0000-0002-5019-2683
Cynthia HawkinsThe Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto (U.T., C.H., S.R.).ORCID 0000-0003-2618-4402
Scott RyallThe Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto (U.T., C.H., S.R.).
Andrés Morales La MadridPediatric Cancer Center Barcelona, Hospital Sant Joan de Deu, Barcelona (A.M.L.M.).
Alvaro LassalettaDepartment of Pediatric Hematology Oncology, Hospital Infantil Universitario Niño Jesús, Madrid (A.L.).
Simon BaileyDepartment of Paediatric Oncology, Sir James Spence Institute of Child Health, Royal Victoria Infirmary Queen, Newcastle upon Tyne (S.B.).
Darren HargravePediatric Oncology Unit, Great Ormond Street Hospital for Children, London (D.H.).
Jason ChiangDepartment of Pathology, St. Jude Children's Research Hospital, Memphis (J.C.).ORCID 0000-0003-3623-8996
Moatasem El-AyadiDepartment of Pediatric Oncology, National Cancer Institute, Cairo University, Cairo (M.E.-A.).
Bruna Minniti MançanoDepartament of Pediatric Oncology, Barretos Cancer Hospital, Barretos (B.M.M.).
Rui Manuel ReisMolecular Oncology Research center, Barretos Cancer Hospital, Barretos (R.M.R.).
Christian HagelInstitute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg (C.H.).ORCID 0000-0003-0518-0824
Hamza GorsiDepartment of Hematology/Oncology, Children's Hospital of Michigan, Detroit (H.G.).
Nicolas SilvestriniClinical Research Platform-Paediatrics, Gynaecology and Obstetrics, Department of Women, Child, and Adolescent Medicine, Geneva University Hospitals and Faculty of Medicine, Geneva (N.S.).
Ahmed GilaniDepartment of Pathology, Children's Hospital Colorado, Aurora (A.G.).
Ludmila PapushaDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow (L.P.).
Paul KlimoDepartment of Surgery, St. Jude Children's Research Hospital, Memphis (P.K.).
Xin ZhouDepartment of Computational Biology, St. Jude Children's Research Hospital, Memphis (C.L., X.Z.).ORCID 0000-0003-3979-8200
Amar GajjarDepartment of Oncology, Division of Neuro-Oncology, St. Jude Children's Research Hospital, Memphis (A.B., S.K.D., A.G., G.W.R.).ORCID 0000-0001-5019-0699
Giles W RobinsonDepartment of Oncology, Division of Neuro-Oncology, St. Jude Children's Research Hospital, Memphis (A.B., S.K.D., A.G., G.W.R.).ORCID 0000-0001-7441-9486
Andre O von BuerenDepartment of Pediatrics, Gynecology and Obstetrics, Division of Pediatric Hematology and Oncology, University Hospital of Geneva, Geneva (L.C., F.T., A.O.v.B.).ORCID 0000-0003-4197-6264

Funding

Viral Vector Technology (VVTSR)P30CA021765 · NCI · ST. JUDE CHILDREN'S RESEARCH HOSPITAL · PI Shondra Michelle Miller · 1985 to 2026
$166.9M
American Lebanese Syrian Associated CharitiesCancer Research UK DRCRPG-Nov21\100002CANSEARCH FoundationCRIS Cancer FoundationDepartment of HealthElse Kröner Excellence FellowshipGreat Ormond Street Hospital Biomedical Research CentreNational Cancer Institute Funds P30CA021765NCI NIH HHS P30 CA021765NHS Foundation TrustNIHRUCL Great Ormond Street Institute of Child HealthUniversity of Geneva
6 · The paper itself

Abstract

backgroundDue to the novelty and rarity of infant-type hemispheric glioma (IHG), optimal treatment and factors determining clinical outcomes are yet to be established.

methodsWe curated a series of 164 patients with IHG; 155 identified by methodical literature search and nine additional patients contributed by collaborators.

resultsAll tumors were hemispheric, diagnosed at a median age of 3.4 (0-52) months, and frequently (95%) non-metastatic. One hundred forty-two (86.5%) tumors harbored fusions involving receptor tyrosine kinase (RTK) genes (ALK [67/142, 47%], NTRK1/2/3 [32/142, 22.5%], ROS1 [29/142, 20.4%], MET [13/142, 9.2%], and ABL2 [1/142, 0.7%]). Sixty-four percentage, 20%, and 8% of patients were treated with surgery and adjuvant chemotherapy, surgery-only, and surgery plus targeted therapy, respectively. Five patients received radiation. Three-year event-free survival (EFS) and overall survival (OS) was 49.5% [40.7-60.2] and 79.6% [72.1-87.9], respectively. Twenty-two patients succumbed to disease, of which tumor progression (8/22, 36%) and intra-cranial hemorrhage (5/22, 23%) were the most common causes. Multivariate analysis showed that the factors most associated with an increased risk of death were no treatment except for surgery and presence of residual tumor after definitive surgery. These findings present a challenging dichotomy where surgery is both a serious risk factor for early death and, when successful, a benefit.

conclusionsTogether, these findings show that IHG is a fusion driven tumor of the very young that is survivable even after progression. While optimal primary therapy for patients with IHG has yet to be established, the findings of this meta-analysis suggest treatment should focus on lowering surgical morbidity and improving its success.

Indexed as

Brain NeoplasmsGliomaChild, PreschoolCombined Modality TherapyFemaleHumansInfantInfant, NewbornMalePrognosisSurvival Ratehigh-grade glioma (HGG)infant-type hemispheric glioma (IHG)pediatric-type diffuse high-grade glioma (pHGG)receptor tyrosine kinase (RTK)tyrosine kinase inhibitor (TKI)

Identifiers

PMID41206756
PMCPMC13070490

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