{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T23:30:07Z","timestamp":1787009407338,"version":"build-2736575974"},"reference-count":165,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2026,1,31]],"date-time":"2026-01-31T00:00:00Z","timestamp":1769817600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Else Kr\u00f6ner-Fresenius Stiftung"},{"name":"Deutsche Forschungsgemeinschaft and the F\u00f6rdergemeinschaft Kinderkrebszentrum Hamburg"},{"name":"German Children\u2019s Cancer Foundation"},{"name":"Fight Kids Cancer"},{"name":"Clinician Scientist program Mildred Scheel Cancer Career Center HaTriCS4 at the University Medical Center Hamburg-Eppendorf, funded by the German Cancer Aid"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,5,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Ependymoma represents a biologically and clinically heterogeneous group of glial tumors that arise throughout the whole neuroaxis and in all age groups. Whereas intracranial ependymoma is usually found in children, adults suffer mostly from spinal cord ependymoma. In comparison to other neuro-oncological tumor entities, ependymoma has been largely understudied for decades. However, the recent years resulted in unprecedented progress with regard to the understanding of the biological underpinnings and clinical features of ependymoma. Here, we review the history of ependymoma research with a focus on the development of classification models throughout the years and a discussion of the most important clinical trials that have led to the current therapeutic standard, comprising maximal safe resection and, in most cases, radiotherapy. Critically, the evidence for effective drugs and chemotherapies in ependymoma is still sparse. However, these important questions may be soon addressed with the finalization of the currently unpublished last generation of multi-institutional trials in Europe (SIOP EP II) and Northern America (ACNS0831). Lastly, we discuss the current challenges in the field of ependymoma research and the necessary next steps toward the goal of findings cures for all types of ependymal tumors.<\/jats:p>","DOI":"10.1093\/neuonc\/noag016","type":"journal-article","created":{"date-parts":[[2026,1,27]],"date-time":"2026-01-27T12:08:33Z","timestamp":1769515713000},"page":"1085-1100","source":"Crossref","is-referenced-by-count":4,"title":["A brief history of ependymoma"],"prefix":"10.1093","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3844-9933","authenticated-orcid":false,"given":"David R","family":"Ghasemi","sequence":"first","affiliation":[{"name":"Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf , Hamburg,","place":["Germany"]},{"name":"Research Institute Children\u2019s Cancer Center Hamburg , Hamburg,","place":["Germany"]},{"name":"Mildred Scheel \u00adCancer Career Center HaTriCS4, University Medical Center Hamburg-Eppendorf , Hamburg,","place":["Germany"]},{"name":"Hopp Children\u2019s Cancer Center Heidelberg (KiTZ) , Heidelberg,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3216-8452","authenticated-orcid":false,"given":"Denise","family":"Obrecht-Sturm","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf , Hamburg,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kimberly M","family":"Wallgren","sequence":"additional","affiliation":[{"name":"Collaborative Ependymoma Research Network (CERN), A Program of the National Brain Tumor Society , Newton, Massachusetts,","place":["USA (K.M.W.)"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin U","family":"Schuhmann","sequence":"additional","affiliation":[{"name":"Section of Pediatric Neurosurgery, Department of Neurosurgery, University Hospital Tuebingen , Tuebingen,","place":["\u00adGermany (M.U.S.)"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beate","family":"Timmermann","sequence":"additional","affiliation":[{"name":"Department of Particle Therapy, University Hospital Essen, West German Proton Therapy Centre Essen (WPE), West German Cancer Center (WTZ) , Essen,","place":["Germany"]},{"name":"German Cancer Consortium (DKTK) , Heidelberg,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5446-9571","authenticated-orcid":false,"given":"Stefan","family":"Rutkowski","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf , Hamburg,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8731-1121","authenticated-orcid":false,"given":"Ulrich","family":"Sch\u00fcller","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf , Hamburg,","place":["Germany"]},{"name":"Research Institute Children\u2019s Cancer Center Hamburg , Hamburg,","place":["Germany"]},{"name":"Institute of Neuropathology, University Medical Center Hamburg-Eppendorf , Hamburg,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3562-6121","authenticated-orcid":false,"given":"Kristian W","family":"Pajtler","sequence":"additional","affiliation":[{"name":"Hopp Children\u2019s Cancer Center Heidelberg (KiTZ) , Heidelberg,","place":["Germany"]},{"name":"Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK) , Heidelberg,","place":["Germany"]},{"name":"Department of Pediatric Oncology, Hematology, Immunology, and Pulmonology, Heidelberg University Hospital , Heidelberg,","place":["Germany"]},{"name":"National Center for Tumor Diseases (NCT) Heidelberg, A Partnership Between DKFZ and Heidelberg University Hospital , Heidelberg,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,1,31]]},"reference":[{"key":"2026051916121986000_noag016-B1","doi-asserted-by":"publisher","first-page":"v1","DOI":"10.1093\/neuonc\/noac202","article-title":"CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2015\u20132019","volume":"24","author":"Ostrom","year":"2022","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B2","doi-asserted-by":"publisher","first-page":"728","DOI":"10.1016\/j.ccell.2015.04.002","article-title":"Molecular classification of ependymal tumors across all CNS compartments, histopathological grades, and age groups","volume":"27","author":"Pajtler","year":"2015","journal-title":"Cancer Cell"},{"key":"2026051916121986000_noag016-B3","doi-asserted-by":"publisher","first-page":"1075","DOI":"10.1007\/s00401-019-02056-2","article-title":"MYCN amplification drives an aggressive form of spinal ependymoma","volume":"138","author":"Ghasemi","year":"2019","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B4","doi-asserted-by":"publisher","first-page":"1231","DOI":"10.1093\/neuonc\/noab106","article-title":"The 2021 WHO classification of tumors of the central nervous system: a summary","volume":"23","author":"Louis","year":"2021","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B5","doi-asserted-by":"publisher","first-page":"878","DOI":"10.1093\/neuonc\/noz058","article-title":"EZHIP\/CXorf67 mimics K27M mutated oncohistones and functions as an intrinsic inhibitor of PRC2 function in aggressive posterior fossa ependymoma","volume":"21","author":"Hubner","year":"2019","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B6","doi-asserted-by":"publisher","first-page":"211","DOI":"10.1007\/s00401-018-1877-0","article-title":"Molecular heterogeneity and CXorf67 alterations in posterior fossa group A (PFA) ependymomas","volume":"136","author":"Pajtler","year":"2018","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B7","doi-asserted-by":"publisher","first-page":"3914","DOI":"10.1038\/s41467-019-11884-5","article-title":"YAP1 subgroup supratentorial ependymoma requires TEAD and nuclear factor I-mediated transcriptional programmes for tumorigenesis","volume":"10","author":"Pajtler","year":"2019","journal-title":"Nat Commun"},{"key":"2026051916121986000_noag016-B8","doi-asserted-by":"publisher","first-page":"208","DOI":"10.1038\/s41568-021-00433-2","article-title":"The biology of ependymomas and emerging novel therapies","volume":"22","author":"Saleh","year":"2022","journal-title":"Nat Rev Cancer"},{"key":"2026051916121986000_noag016-B9","doi-asserted-by":"publisher","first-page":"267","DOI":"10.1093\/neuonc\/noae064.203","article-title":"Distinct relapse pattern across molecular ependymoma types","volume":"27","author":"Obrecht-Sturm","year":"2025","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B10","doi-asserted-by":"publisher","first-page":"440","DOI":"10.1093\/neuonc\/nou162","article-title":"Clinical course and progression-free survival of adult intracranial and spinal ependymoma patients","volume":"17","author":"Vera-Bolanos","year":"2015","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B11","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1007\/s11060-021-03867-8","article-title":"Systemic chemotherapy of pediatric recurrent ependymomas: results from the german HIT-REZ studies","volume":"155","author":"Adolph","year":"2021","journal-title":"J Neurooncol"},{"key":"2026051916121986000_noag016-B12","doi-asserted-by":"publisher","first-page":"1012","DOI":"10.1093\/neuonc\/noaa276","article-title":"Local and systemic therapy of recurrent ependymoma in children and adolescents: short- and long-term results of the E-HIT-REZ 2005 study","volume":"23","author":"Adolph","year":"2021","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B13","doi-asserted-by":"publisher","first-page":"563","DOI":"10.1007\/s003810050544","article-title":"Chemotherapy for intracranial ependymomas","volume":"15","author":"Bouffet","year":"1999","journal-title":"Childs Nerv Syst"},{"key":"2026051916121986000_noag016-B14","doi-asserted-by":"publisher","first-page":"287","DOI":"10.1186\/s12885-016-2323-0","article-title":"Chemotherapy for intracranial ependymoma in adults","volume":"16","author":"Gramatzki","year":"2016","journal-title":"BMC Cancer"},{"key":"2026051916121986000_noag016-B15","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1007\/s00401-016-1643-0","article-title":"The current consensus on the clinical management of intracranial ependymoma and its distinct molecular variants","volume":"133","author":"Pajtler","year":"2017","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B16","doi-asserted-by":"publisher","first-page":"e31275","DOI":"10.1002\/pbc.31275","article-title":"The medical and functional burden of surviving childhood ependymoma: a population-based study in Ontario, Canada","volume":"71","author":"Coltin","year":"2024","journal-title":"Pediatr Blood Cancer"},{"key":"2026051916121986000_noag016-B17","doi-asserted-by":"publisher","first-page":"2113","DOI":"10.1093\/neuonc\/noae092","article-title":"Risk factors for domain-specific neurocognitive outcome in pediatric survivors of a brain tumor in the posterior fossa \u2013 results of the HIT 2000 trial","volume":"26","author":"Mynarek","year":"2024","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B18","doi-asserted-by":"crossref","first-page":"1320369","DOI":"10.3389\/fncel.2023.1320369","article-title":"History of research concerning the ependyma: a view from inside the human brain","volume":"17","author":"Bigio","year":"2024","journal-title":"Front Cell Neurosci"},{"key":"2026051916121986000_noag016-B19","volume-title":"Die krankhaften Geschw\u00fclste: dreissig Vorlesungen gehalten w\u00e4hrend des Wintersemesters 1862-1863 an der Universit\u00e4t zu Berlin","author":"Virchow","year":"1865"},{"key":"2026051916121986000_noag016-B20","first-page":"127","article-title":"Ueber die pathologisch-anatomischen Vorg\u00e4nge am St\u00fctzger\u00fcst des Centralnervensystems","volume":"157","author":"Storch","year":"1899","journal-title":"Archiv f\u00fcr pathologische Anatomie und Physiologie und f\u00fcr klinische Medicin"},{"key":"2026051916121986000_noag016-B21","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1001\/archinte.1908.000500700950091","article-title":"Three gliomata of ependymal origin; two in the fourth ventricle, one subcutaneous over the coccyx","volume":"8","author":"Mallory","year":"1902","journal-title":"J Med Res"},{"key":"2026051916121986000_noag016-B22","first-page":"43","article-title":"\u00dcber ein Gliageschwulst des IV. Ventrikels (Neuroepithelioma gliomatosum columnocellulare veli medullaris posterioris) nebst allgemeinen Bemerkungen \u00fcber die Gliome \u00fcberhaupt. Beitr\u00e4ge path","volume":"34","author":"Muthmann","year":"1903","journal-title":"Anat"},{"key":"2026051916121986000_noag016-B23","doi-asserted-by":"publisher","first-page":"476","DOI":"10.1055\/s-0028-1139190","article-title":"Pathologisch-anatomische Bemerkungen zu dem zweiten vorstehenden Fall A. Fraenkel\u2019s","volume":"24","author":"Benda","year":"1898","journal-title":"Dtsch med Wochenschr"},{"key":"2026051916121986000_noag016-B24","first-page":"627","article-title":"Ependymomas of the lateral and fourth ventricles of the brain","volume":"1","author":"Hirsch","year":"1925","journal-title":"Am J Pathol"},{"key":"2026051916121986000_noag016-B25","volume-title":"A Classification of the Tumors of the Glioma Group on a Histogenetic Basis with a Correlated Study of Prognosis","author":"Bailey","year":"1926"},{"key":"2026051916121986000_noag016-B26","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1001\/archneurpsyc.1924.02190310007001","article-title":"A study of tumors arising from ependymal cells","volume":"11","author":"Bailey","year":"1924","journal-title":"Arch NeurPsych"},{"key":"2026051916121986000_noag016-B27","first-page":"354","article-title":"Further remarks concerning tumors of the glioma group","volume":"40","author":"Bailey","year":"1927","journal-title":"Bull Johns Hopkins Hosp"},{"key":"2026051916121986000_noag016-B28","doi-asserted-by":"publisher","first-page":"1536","DOI":"10.1002\/1097-0142(19850401)","article-title":"Ependymoblastoma. A reappraisal of a rare embryonal tumor","volume":"55","author":"Mork","year":"1985","journal-title":"Cancer"},{"key":"2026051916121986000_noag016-B29","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1097\/00005072-197201000-00002","article-title":"Cytogenesis and differentiation of primitive central neuroepithelial tumors","volume":"31","author":"Rubinstein","year":"1972","journal-title":"J Neuropathol Exp Neurol"},{"key":"2026051916121986000_noag016-B30","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1002\/1097-0142(19850401)","article-title":"The definition of the ependymoblastoma","volume":"90","author":"Rubinstein","year":"1970","journal-title":"Arch Pathol"},{"key":"2026051916121986000_noag016-B31","doi-asserted-by":"publisher","first-page":"133","DOI":"10.1111\/j.1750-3639.2008.00253.x","article-title":"Ependymoblastoma: dear, damned, distracting diagnosis, farewell!*","volume":"20","author":"Judkins","year":"2010","journal-title":"Brain Pathol"},{"key":"2026051916121986000_noag016-B32","volume-title":"WHO Classification of Tumours of the Central Nervous System","author":"Louis","year":"2016","edition":"4 \u00fc"},{"key":"2026051916121986000_noag016-B33","first-page":"27","article-title":"Ependymomas: study of109 cases","volume":"16","author":"Kernohan","year":"1938","journal-title":"Res Publ Assoc Nerv Ment Dis"},{"key":"2026051916121986000_noag016-B34","doi-asserted-by":"publisher","first-page":"1281","DOI":"10.1001\/archneurpsyc.1932.02230180010002","article-title":"Histologic classification of tumors of the central nervous system","volume":"27","author":"Roussy","year":"1932","journal-title":"Arch NeurPsych"},{"key":"2026051916121986000_noag016-B35","first-page":"45","article-title":"Essai de classification des tumeurs c\u00e9r\u00e9brales","volume":"1","author":"Roussy","year":"1924","journal-title":"Ann Anat Path"},{"key":"2026051916121986000_noag016-B36","first-page":"993","volume-title":"Cytology and Cellular Pathology of the Nervous System","author":"Kernohan","year":"1932"},{"key":"2026051916121986000_noag016-B37","doi-asserted-by":"publisher","first-page":"232","DOI":"10.3171\/jns.1945.2.3.0232","article-title":"Subependymoma: a newly recognized tumor of subependymal derivation","volume":"2","author":"Scheinker","year":"1945","journal-title":"J Neurosurg"},{"key":"2026051916121986000_noag016-B38","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/S0025-6196(25)26181-X","article-title":"Ependymomas","volume":"24","author":"Mabon","year":"1949","journal-title":"Proc Staff Meet Mayo Clin"},{"key":"2026051916121986000_noag016-B39","first-page":"24","article-title":"Das Ependymom der Gro\u00dfhirnhemisph\u00e4ren im Jugendalter","volume":"2","author":"T\u00f6nnis","year":"1937","journal-title":"Zentralblatt f\u00fcr Neurochirurgie"},{"key":"2026051916121986000_noag016-B40","volume-title":"Die Hirngeschw\u00fclste in biologischer und morphologischer Darstellung","author":"Z\u00fclch","year":"1958"},{"key":"2026051916121986000_noag016-B41","volume-title":"Histological Typing of Tumours of the Central Nervous System","author":"Z\u00fclch","year":"1979","edition":"1st ed"},{"key":"2026051916121986000_noag016-B42","doi-asserted-by":"publisher","first-page":"215","DOI":"10.1093\/jnen\/61.3.215","article-title":"The WHO classification of tumors of the nervous system","volume":"61","author":"Kleihues","year":"2002","journal-title":"J Neuropathol Exp Neurol"},{"key":"2026051916121986000_noag016-B43","volume-title":"World Health Organization Classification of Tumours of the Nervous System","author":"Kleihues","year":"2000","edition":"3rd ed"},{"key":"2026051916121986000_noag016-B44","doi-asserted-by":"crossref","DOI":"10.1007\/978-3-642-84988-6","volume-title":"Histological Typing of Tumours of the Central Nervous System","author":"Kleihues","year":"1993","edition":"2nd ed"},{"key":"2026051916121986000_noag016-B45","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1186\/1477-5751-10-7","article-title":"Histopathological grading of pediatric ependymoma: reproducibility and clinical relevance in European trial cohorts","volume":"10","author":"Ellison","year":"2011","journal-title":"J Negat Results Biomed"},{"key":"2026051916121986000_noag016-B46","volume-title":"WHO Classification of Tumours of the Central Nervous System","author":"Louis","year":"2007","edition":"4th ed"},{"key":"2026051916121986000_noag016-B47","doi-asserted-by":"publisher","first-page":"323","DOI":"10.1016\/j.ccr.2005.09.001","article-title":"Radial glia cells are candidate stem cells of ependymoma","volume":"8","author":"Taylor","year":"2005","journal-title":"Cancer Cell"},{"key":"2026051916121986000_noag016-B48","doi-asserted-by":"publisher","first-page":"143","DOI":"10.1016\/j.ccr.2011.07.007","article-title":"Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma","volume":"20","author":"Witt","year":"2011","journal-title":"Cancer Cell"},{"key":"2026051916121986000_noag016-B49","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1038\/nature13109","article-title":"C11orf95-RELA fusions drive oncogenic NF-kappaB signalling in ependymoma","volume":"506","author":"Parker","year":"2014","journal-title":"Nature"},{"key":"2026051916121986000_noag016-B50","doi-asserted-by":"publisher","first-page":"827","DOI":"10.18632\/oncoscience.202","article-title":"Molecular dissection of ependymomas","volume":"2","author":"Pajtler","year":"2015","journal-title":"Oncoscience"},{"key":"2026051916121986000_noag016-B51","doi-asserted-by":"publisher","first-page":"205","DOI":"10.1111\/bpa.12659","article-title":"Childhood supratentorial ependymomas with YAP1-MAMLD1 fusion: an entity with characteristic clinical, radiological, cytogenetic and histopathological features","volume":"29","author":"Andreiuolo","year":"2019","journal-title":"Brain Pathol"},{"key":"2026051916121986000_noag016-B52","doi-asserted-by":"publisher","first-page":"919","DOI":"10.1093\/neuonc\/now025","article-title":"Supratentorial clear cell ependymomas with branching capillaries demonstrate characteristic clinicopathological features and pathological activation of nuclear factor-kappaB signaling","volume":"18","author":"Figarella-Branger","year":"2016","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B53","doi-asserted-by":"publisher","first-page":"305","DOI":"10.1007\/s00401-019-02090-0","article-title":"Molecular characterization of histopathological ependymoma variants","volume":"139","author":"Neumann","year":"2020","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B54","doi-asserted-by":"publisher","first-page":"803","DOI":"10.1007\/s00401-016-1545-1","article-title":"The 2016 World Health \u00adOrganization classification of tumors of the central nervous system: a summary","volume":"131","author":"Louis","year":"2016","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B55","doi-asserted-by":"publisher","first-page":"443","DOI":"10.1097\/CCO.0000000000000411","article-title":"Put away your microscopes: the ependymoma molecular era has begun","volume":"29","author":"Mack","year":"2017","journal-title":"Curr Opin Oncol"},{"key":"2026051916121986000_noag016-B56","doi-asserted-by":"publisher","first-page":"227","DOI":"10.1007\/s00401-018-1888-x","article-title":"Heterogeneity within the PF-EPN-B ependymoma subgroup","volume":"136","author":"Cavalli","year":"2018","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B57","doi-asserted-by":"publisher","first-page":"373","DOI":"10.1007\/s00401-022-02435-2","article-title":"Recurrent ACVR1 mutations in posterior fossa ependymoma","volume":"144","author":"Pratt","year":"2022","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B58","doi-asserted-by":"publisher","first-page":"2146","DOI":"10.1038\/s41467-019-09981-6","article-title":"PFA ependymoma-associated protein EZHIP inhibits PRC2 activity through a H3 K27M-like mechanism","volume":"10","author":"Jain","year":"2019","journal-title":"Nat Commun"},{"key":"2026051916121986000_noag016-B59","doi-asserted-by":"publisher","first-page":"366ra161","DOI":"10.1126\/scitranslmed.aah6904","article-title":"Lowered H3K27me3 and DNA hypomethylation define poorly prognostic pediatric posterior fossa ependymomas","volume":"8","author":"Bayliss","year":"2016","journal-title":"Sci Transl Med"},{"key":"2026051916121986000_noag016-B60","doi-asserted-by":"crossref","first-page":"e70035","DOI":"10.1111\/bpa.70035","article-title":"cIMPACT-NOW update 11: proposal on adaptation of diagnostic criteria for IDH- and H3-wildtype diffuse high-grade gliomas and for posterior fossa ependymal tumors","volume":"36","author":"Wesseling","year":"2026","journal-title":"Brain Pathol"},{"key":"2026051916121986000_noag016-B61","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1186\/s40478-020-00973-y","article-title":"High level MYCN amplification and distinct methylation signature define an aggressive subtype of spinal cord ependymoma","volume":"8","author":"Raffeld","year":"2020","journal-title":"Acta Neuropathol Commun"},{"key":"2026051916121986000_noag016-B62","doi-asserted-by":"publisher","first-page":"791","DOI":"10.1093\/jnen\/nlz064","article-title":"Spinal cord ependymomas with MYCN amplification show aggressive clinical behavior","volume":"78","author":"Swanson","year":"2019","journal-title":"J Neuropathol Exp Neurol"},{"key":"2026051916121986000_noag016-B63","doi-asserted-by":"publisher","first-page":"2230","DOI":"10.1158\/2159-8290.CD-20-0963","article-title":"Cross-species genomics reveals oncogenic dependencies in ZFTA\/C11orf95 fusion-positive supratentorial ependymomas","volume":"11","author":"Zheng","year":"2021","journal-title":"Cancer Discov"},{"key":"2026051916121986000_noag016-B64","doi-asserted-by":"publisher","first-page":"455","DOI":"10.1007\/s00401-020-02260-5","article-title":"Supratentorial ependymoma in childhood: more than just RELA or YAP","volume":"141","author":"Zschernack","year":"2021","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B65","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1186\/s40478-021-01238-y","article-title":"Supratentorial non-RELA, ZFTA-fused ependymomas: a comprehensive phenotype genotype correlation highlighting the number of zinc fingers in ZFTA-NCOA1\/2 fusions","volume":"9","author":"Tauzi\u00e8de-Espariat","year":"2021","journal-title":"Acta Neuropathol Commun"},{"key":"2026051916121986000_noag016-B66","doi-asserted-by":"publisher","first-page":"134","DOI":"10.1186\/s40478-018-0630-1","article-title":"Significance of molecular classification of ependymomas: C11orf95-RELA fusion-negative supratentorial ependymomas are a heterogeneous group of tumors","volume":"6","author":"Fukuoka","year":"2018","journal-title":"Acta Neuropathol Commun"},{"key":"2026051916121986000_noag016-B67","doi-asserted-by":"publisher","first-page":"2216","DOI":"10.1158\/2159-8290.CD-20-1052","article-title":"ZFTA-translocations constitute ependymoma chromatin remodeling and transcription factors","volume":"11","author":"Kupp","year":"2021","journal-title":"Cancer Discov"},{"key":"2026051916121986000_noag016-B68","doi-asserted-by":"publisher","first-page":"2200","DOI":"10.1158\/2159-8290.CD-20-1066","article-title":"ZFTA-RELA dictates oncogenic transcriptional programs to drive aggressive supratentorial ependymoma","volume":"11","author":"Arabzade","year":"2021","journal-title":"Cancer Discov"},{"key":"2026051916121986000_noag016-B69","doi-asserted-by":"publisher","first-page":"1360","DOI":"10.1093\/neuonc\/noab034","article-title":"Ultra high-risk PFA ependymoma is characterized by loss of chromosome 6q","volume":"23","author":"Baroni","year":"2021","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B70","doi-asserted-by":"publisher","first-page":"2001","DOI":"10.1158\/1078-0432.CCR-11-2489","article-title":"Copy number gain of 1q25 predicts poor progression-free survival for pediatric intracranial ependymomas and enables patient risk stratification: a prospective European clinical trial cohort analysis on behalf of the children\u2019s cancer leukaemia group (CCLG), Societe Francaise d\u2018Oncologie Pediatrique (SFOP), and international society for pediatric oncology (SIOP)","volume":"18","author":"Kilday","year":"2012","journal-title":"Clin Cancer Res"},{"key":"2026051916121986000_noag016-B71","doi-asserted-by":"publisher","first-page":"3182","DOI":"10.1200\/JCO.2009.27.3359","article-title":"Molecular staging of intracranial ependymoma in children and adults","volume":"28","author":"Korshunov","year":"2010","journal-title":"J Clin Oncol"},{"key":"2026051916121986000_noag016-B72","doi-asserted-by":"publisher","first-page":"2070","DOI":"10.1158\/1078-0432.CCR-05-2363","article-title":"Identification of gains on 1q and epidermal growth factor receptor overexpression as independent prognostic markers in intracranial ependymoma","volume":"12","author":"Mendrzyk","year":"2006","journal-title":"Clin Cancer Res"},{"key":"2026051916121986000_noag016-B73","doi-asserted-by":"publisher","first-page":"405","DOI":"10.1007\/s00401-020-02169-z","article-title":"CDKN2A deletion in supratentorial ependymoma with RELA alteration indicates a dismal prognosis: a retrospective analysis of the HIT ependymoma trial cohort","volume":"140","author":"J\u00fcnger","year":"2020","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B74","doi-asserted-by":"publisher","first-page":"929","DOI":"10.1038\/sj.bjc.6600180","article-title":"Genetic abnormalities detected in ependymomas by comparative genomic hybridisation","volume":"86","author":"Carter","year":"2002","journal-title":"Br J Cancer"},{"key":"2026051916121986000_noag016-B75","doi-asserted-by":"publisher","first-page":"705","DOI":"10.1007\/s00401-017-1752-4","article-title":"Immunohistochemical analysis of H3K27me3 demonstrates global reduction in group\u2014a childhood posterior fossa ependymoma and is a powerful predictor of outcome","volume":"134","author":"Panwalkar","year":"2017","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B76","doi-asserted-by":"publisher","first-page":"82","DOI":"10.1093\/jnen\/nlab105","article-title":"L1CAM is not a reliable diagnostic biomarker for distinguishing supratentorial ependymomas, ZFTA fusion-positive from other central nervous system tumors","volume":"81","author":"Aboubakr","year":"2022","journal-title":"J Neuropathol Exp Neurol"},{"key":"2026051916121986000_noag016-B77","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1177\/1066896918800812","article-title":"L1CAM immunopositivity in anaplastic supratentorial ependymomas: correlation with clinical and \u00adhistological parameters","volume":"27","author":"Chavali","year":"2019","journal-title":"Int J Surg Pathol"},{"key":"2026051916121986000_noag016-B78","doi-asserted-by":"publisher","first-page":"56","DOI":"10.1097\/PAS.0000000000000979","article-title":"Role of immunohistochemistry in the identification of supratentorial C11ORF95-RELA fused ependymoma in routine neuropathology","volume":"43","author":"Gessi","year":"2019","journal-title":"Am J Surg Pathol"},{"key":"2026051916121986000_noag016-B79","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1007\/s11060-018-2767-y","article-title":"C11orf95-RELA fusions and upregulated NF-KB signalling characterise a subset of aggressive supratentorial ependymomas that express L1CAM and nestin","volume":"138","author":"Malgulwar","year":"2018","journal-title":"J Neurooncol"},{"key":"2026051916121986000_noag016-B80","doi-asserted-by":"crossref","first-page":"1871","DOI":"10.1093\/neuonc\/noad055","article-title":"Optimising biomarkers for accurate ependymoma diagnosis, prognostication and stratification within International Clinical Trials: a BIOMECA study","volume":"10","author":"Chapman","year":"2023","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B81","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1007\/s00401-025-02866-7","article-title":"Strong nuclear expression of HOXB13 is a reliable surrogate marker for DNA methylome profiling to distinguish myxopapillary ependymoma from spinal ependymoma","volume":"149","author":"Purkait","year":"2025","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B82","doi-asserted-by":"publisher","first-page":"1689","DOI":"10.1093\/neuonc\/noac088","article-title":"Comprehensive profiling of myxopapillary ependymomas identifies a distinct molecular subtype with relapsing disease","volume":"24","author":"Bockmayr","year":"2022","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B83","doi-asserted-by":"publisher","first-page":"1215","DOI":"10.1093\/neuonc\/noab120","article-title":"The 2021 WHO classification of tumors of the central nervous system: clinical implications","volume":"23","author":"Wen","year":"2021","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B84","volume-title":"World Health Organization Classification of Tumours of the Central Nervous System","author":"WHO Classification of Tumours Editorial Board","year":"2021","edition":"5th ed"},{"key":"2026051916121986000_noag016-B85","doi-asserted-by":"publisher","first-page":"469","DOI":"10.1038\/nature26000","article-title":"DNA methylation-based classification of central nervous system tumours","volume":"555","author":"Capper","year":"2018","journal-title":"Nature"},{"key":"2026051916121986000_noag016-B86","doi-asserted-by":"publisher","first-page":"22","DOI":"10.1007\/s00401-023-02668-9","article-title":"Transcriptomic and epigenetic dissection of spinal ependymoma (SP-EPN) identifies clinically relevant subtypes enriched for tumors with and without NF2 mutation","volume":"147","author":"Neyazi","year":"2024","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B87","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/noaf210","article-title":"Single nucleus transcriptomics of spinal ependymoma subtypes recognizes intratumoral heterogeneity","author":"Hack","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B88","doi-asserted-by":"publisher","first-page":"83","DOI":"10.1007\/s00401-024-02740-y","article-title":"Anaplastic histology and distinct molecular features in a small series of spinal cord ependymomas","volume":"147","author":"Schuller","year":"2024","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B89","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/noaf251","article-title":"Integrated transcriptomic landscape of medulloblastoma and ependymoma reveals novel tumor subtype-specific biology","author":"Arora","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B90","doi-asserted-by":"publisher","first-page":"959","DOI":"10.1007\/s00401-021-02300-8","article-title":"TERT promoter mutation and chromosome 6 loss define a high-risk subtype of ependymoma evolving from posterior fossa subependymoma","volume":"141","author":"Thomas","year":"2021","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B91","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1007\/s00401-023-02674-x","article-title":"Molecular characteristics and improved survival prediction in a cohort of 2023 ependymomas","volume":"147","author":"Pohl","year":"2024","journal-title":"Acta Neuropathol"},{"key":"2026051916121986000_noag016-B92","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/noaf218","article-title":"Developing an advanced risk stratification model for pediatric intracranial ependymoma based on the prospective trial E-HIT2000 and subsequent registries [published online ahead of print September 30, 2025]","author":"von Hoff","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B93","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1186\/s40478-024-01921-w","article-title":"Biglycan-driven risk stratification in ZFTA-RELA fusion supratentorial ependymomas through transcriptome profiling","volume":"13","author":"Okonechnikov","year":"2025","journal-title":"Acta Neuropathol Commun"},{"issue":"6","key":"2026051916121986000_noag016-B94","doi-asserted-by":"crossref","first-page":"e70018","DOI":"10.1111\/bpa.70018","article-title":"cIMPACT-NOW update 10: recommendations for defining new types for central nervous system tumor classification","volume":"35","author":"Hawkins","year":"2025","journal-title":"Brain Pathol"},{"key":"2026051916121986000_noag016-B95","doi-asserted-by":"publisher","first-page":"936","DOI":"10.1093\/neuonc\/noac012","article-title":"SIOP ependymoma I: final results, long term follow-up and molecular analysis of the trial cohort: a BIOMECA consortium study","volume":"24","author":"Ritzmann","year":"2022","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B96","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1002\/(SICI)1096-911X(199607)27:1&lt;8::AID-MPO3&gt;3.0.CO;2-K","article-title":"Adjuvant chemotherapy of childhood posterior fossa ependymoma: cranio-spinal irradiation with or without adjuvant CCNU, vincristine, and prednisone: a childrens cancer group study","volume":"27","author":"Evans","year":"1996","journal-title":"Med Pediatr Oncol"},{"key":"2026051916121986000_noag016-B97","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1002\/pbc.20935","article-title":"Outcome for young children newly diagnosed with ependymoma, treated with intensive induction chemotherapy followed by myeloablative chemotherapy and autologous stem cell rescue","volume":"49","author":"Zacharoulis","year":"2007","journal-title":"Pediatr Blood Cancer"},{"key":"2026051916121986000_noag016-B98","doi-asserted-by":"publisher","first-page":"278","DOI":"10.1016\/j.radonc.2005.10.016","article-title":"Role of radiotherapy in anaplastic ependymoma in children under age of 3 years: results of the prospective german brain tumor trials HIT-SKK 87 and 92","volume":"77","author":"Timmermann","year":"2005","journal-title":"Radiother Oncol"},{"key":"2026051916121986000_noag016-B99","doi-asserted-by":"publisher","first-page":"319","DOI":"10.1002\/(SICI)1096-911X(199806)","article-title":"Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation","volume":"30","author":"Bouffet","year":"1998","journal-title":"Med Pediatr Oncol"},{"key":"2026051916121986000_noag016-B100","doi-asserted-by":"publisher","first-page":"842","DOI":"10.1200\/JCO.2002.20.3.842","article-title":"Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective german brain tumor trials HIT 88\/89 and 91","volume":"20","author":"Timmermann","year":"2002","journal-title":"J Clin Oncol"},{"key":"2026051916121986000_noag016-B101","doi-asserted-by":"publisher","first-page":"1725","DOI":"10.1056\/NEJM199306173282401","article-title":"Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors","volume":"328","author":"Duffner","year":"1993","journal-title":"N Engl J Med"},{"key":"2026051916121986000_noag016-B102","doi-asserted-by":"publisher","first-page":"1607","DOI":"10.1200\/JCO.1994.12.8.1607","article-title":"Survival of infants with primitive neuroectodermal tumors or malignant ependymomas of the CNS treated with eight drugs in 1 day: a report from the childrens cancer group","volume":"12","author":"Geyer","year":"1994","journal-title":"J Clin Oncol"},{"key":"2026051916121986000_noag016-B103","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1097\/00043426-199803000-00007","article-title":"Postoperative chemotherapy in children less than 4 years of age with malignant brain tumors: promising initial response to a VETOPEC-based regimen. A study of the Australian and New Zealand children\u2019s cancer study group (ANZCCSG)","volume":"20","author":"White","year":"1998","journal-title":"J Pediatr Hematol Oncol"},{"key":"2026051916121986000_noag016-B104","doi-asserted-by":"publisher","first-page":"1288","DOI":"10.1200\/JCO.2001.19.5.1288","article-title":"Postoperative chemotherapy without irradiation for ependymoma in children under 5 years of age: a multicenter trial of the french society of pediatric oncology","volume":"19","author":"Grill","year":"2001","journal-title":"J Clin Oncol"},{"key":"2026051916121986000_noag016-B105","doi-asserted-by":"publisher","first-page":"1336","DOI":"10.1016\/j.ijrobp.2003.08.030","article-title":"Hyperfractionated radiotherapy and chemotherapy for childhood ependymoma: final results of the first prospective AIEOP (associazione italiana di Ematologia-Oncologia pediatrica) study","volume":"58","author":"Massimino","year":"2004","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"2026051916121986000_noag016-B106","doi-asserted-by":"publisher","first-page":"3156","DOI":"10.1200\/JCO.2004.11.142","article-title":"Preliminary results from a phase II trial of conformal radiation therapy and evaluation of radiation-related CNS effects for pediatric patients with localized ependymoma","volume":"22","author":"Merchant","year":"2004","journal-title":"J Clin Oncol"},{"key":"2026051916121986000_noag016-B107","doi-asserted-by":"publisher","first-page":"629","DOI":"10.3171\/jns.2005.102.4.0629","article-title":"Is gross-total resection sufficient treatment for posterior fossa ependymomas?","volume":"102","author":"Rogers","year":"2005","journal-title":"J Neurosurg"},{"key":"2026051916121986000_noag016-B108","doi-asserted-by":"publisher","first-page":"258","DOI":"10.1016\/s1470-2045(08)70342-5","article-title":"Conformal radiotherapy after surgery for paediatric ependymoma: a prospective study","volume":"10","author":"Merchant","year":"2009","journal-title":"Lancet Oncol"},{"key":"2026051916121986000_noag016-B109","doi-asserted-by":"crossref","first-page":"1183","DOI":"10.1002\/pbc.24274","article-title":"Phase II study of pre-irradiation chemotherapy for childhood intracranial ependymoma. Children\u2019s cancer group protocol 9942: a report from the children\u2019s oncology group","volume":"59","author":"Garvin","year":"2012","journal-title":"Pediatr Blood Cancer"},{"key":"2026051916121986000_noag016-B110","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1111\/j.1600-0463.1930.tb06503.x","article-title":"Further notes on the cerebellar medulloblastomas: the effect of roentgen radiation","volume":"6","author":"Bailey","year":"1930","journal-title":"Am J Pathol"},{"key":"2026051916121986000_noag016-B111","doi-asserted-by":"publisher","first-page":"323","DOI":"10.3109\/00016925309136718","article-title":"Cerebellar medulloblastoma: treatment by irradiation of the whole central nervous system","volume":"39","author":"Paterson","year":"1953","journal-title":"Acta Radiol (Stockh)"},{"key":"2026051916121986000_noag016-B112","doi-asserted-by":"publisher","first-page":"287","DOI":"10.1016\/s0360-3016(99)00414-9","article-title":"Combined postoperative irradiation and chemotherapy for anaplastic ependymomas in childhood: results of the german prospective trials HIT 88\/89 and HIT 91","volume":"46","author":"Timmermann","year":"2000","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"2026051916121986000_noag016-B113","doi-asserted-by":"publisher","first-page":"469","DOI":"10.1007\/s00066-002-0971-2","article-title":"Die interdisziplin\u00e4re Therapie von Ependymomen im Kindesalter","volume":"178","author":"Timmermann","year":"2002","journal-title":"Strahlentherapie und Onkologie"},{"key":"2026051916121986000_noag016-B114","doi-asserted-by":"publisher","first-page":"848","DOI":"10.1093\/neuonc\/noaa257","article-title":"Second series by the Italian association of pediatric hematology and oncology of children and adolescents with intracranial ependymoma: an integrated molecular and clinical characterization with a long-term follow-up","volume":"23","author":"Massimino","year":"2021","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B115","doi-asserted-by":"publisher","first-page":"1193","DOI":"10.1093\/neuonc\/noab301","article-title":"Evaluation of dose, volume, and outcome in children with localized, intracranial ependymoma treated with proton therapy within the prospective KiProReg Study","volume":"24","author":"Peters","year":"2022","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B116","doi-asserted-by":"publisher","first-page":"644","DOI":"10.1080\/0284186X.2017.1413248","article-title":"Outcomes following proton therapy for pediatric ependymoma","volume":"57","author":"Indelicato","year":"2018","journal-title":"Acta Oncol"},{"key":"2026051916121986000_noag016-B117","doi-asserted-by":"publisher","first-page":"1546","DOI":"10.1016\/j.ijrobp.2005.05.028","article-title":"Radiation dosimetry predicts IQ after conformal radiation therapy in pediatric patients with localized ependymoma","volume":"63","author":"Merchant","year":"2005","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"2026051916121986000_noag016-B118","doi-asserted-by":"publisher","first-page":"1451","DOI":"10.1093\/neuonc\/now108","article-title":"Final results of the second prospective AIEOP protocol for pediatric intracranial ependymoma","volume":"18","author":"Massimino","year":"2016","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B119","doi-asserted-by":"publisher","first-page":"974","DOI":"10.1200\/JCO.18.01765","article-title":"Conformal radiation therapy for pediatric ependymoma, chemotherapy for incompletely resected ependymoma, and observation for completely resected, supratentorial ependymoma","volume":"37","author":"Merchant","year":"2019","journal-title":"J Clin Oncol"},{"key":"2026051916121986000_noag016-B120","doi-asserted-by":"publisher","first-page":"696","DOI":"10.1016\/S1470-2045(07)70208-5","article-title":"Primary postoperative chemotherapy without radiotherapy for intracranial ependymoma in children: the UKCCSG\/SIOP prospective study","volume":"8","author":"Grundy","year":"2007","journal-title":"Lancet Oncol"},{"key":"2026051916121986000_noag016-B121","doi-asserted-by":"publisher","first-page":"367","DOI":"10.1007\/s11060-011-0671-9","article-title":"Pediatric intracranial ependymoma: the roles of surgery, radiation and chemotherapy","volume":"106","author":"Pejavar","year":"2012","journal-title":"J Neurooncol"},{"key":"2026051916121986000_noag016-B122","doi-asserted-by":"publisher","first-page":"673","DOI":"10.3171\/2013.2.PEDS12345","article-title":"A systematic review of treatment outcomes in pediatric patients with intracranial ependymomas","volume":"11","author":"Cage","year":"2013","journal-title":"J Neurosurg Pediatr"},{"key":"2026051916121986000_noag016-B123","doi-asserted-by":"publisher","first-page":"457","DOI":"10.1093\/neuonc\/not163","article-title":"Benefit from prolonged dose-intensive chemotherapy for infants with malignant brain tumors is restricted to patients with ependymoma: a report of the pediatric oncology group randomized controlled trial 9233\/34","volume":"16","author":"Strother","year":"2014","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B124","doi-asserted-by":"publisher","first-page":"1620","DOI":"10.1093\/neuonc\/nov181","article-title":"Phase I study of 5-fluorouracil in children and young adults with recurrent ependymoma","volume":"17","author":"Wright","year":"2015","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B125","doi-asserted-by":"publisher","first-page":"1544","DOI":"10.1158\/1078-0432.CCR-23-3156","article-title":"Development of chromosome 1q+ specific treatment for highest risk pediatric posterior fossa ependymoma","volume":"30","author":"Griesinger","year":"2024","journal-title":"Clin Cancer Res"},{"key":"2026051916121986000_noag016-B126","doi-asserted-by":"publisher","first-page":"vdad011","DOI":"10.1093\/noajnl\/vdad011","article-title":"Phase II study of everolimus for recurrent or progressive pediatric ependymoma","volume":"5","author":"Bowers","year":"2023","journal-title":"Neurooncol Adv"},{"key":"2026051916121986000_noag016-B127","doi-asserted-by":"publisher","first-page":"468","DOI":"10.1093\/neuonc\/noaa240","article-title":"A phase II study of dose-dense temozolomide and lapatinib for recurrent low-grade and anaplastic supratentorial, infratentorial, and spinal cord ependymoma","volume":"23","author":"Gilbert","year":"2021","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B128","doi-asserted-by":"publisher","first-page":"267","DOI":"10.1093\/neuonc\/noae166","article-title":"Distinct relapse pattern across molecular ependymoma types","volume":"27","author":"Obrecht-Sturm","year":"2025","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B129","doi-asserted-by":"publisher","first-page":"166","DOI":"10.1016\/j.ijrobp.2018.05.036","article-title":"Pediatric localized intracranial ependymomas: a multicenter analysis of the Soci\u00e9t\u00e9 Fran\u00e7aise de lutte contre les Cancers de l\u2018Enfant (SFCE) from 2000 to 2013","volume":"102","author":"Ducassou","year":"2018","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"2026051916121986000_noag016-B130","doi-asserted-by":"publisher","first-page":"e28426","DOI":"10.1002\/pbc.28426","article-title":"A retrospective analysis of recurrent pediatric ependymoma reveals extremely poor survival and ineffectiveness of current treatments across central nervous system locations and molecular subgroups","volume":"67","author":"Ritzmann","year":"2020","journal-title":"Pediatr Blood Cancer"},{"key":"2026051916121986000_noag016-B131","doi-asserted-by":"publisher","first-page":"2114","DOI":"10.1002\/ijc.70016","article-title":"How long should survivors of pediatric medulloblastoma and ependymoma be screened for recurrence? A retrospective cohort study","volume":"157","author":"Meulendijks","year":"2025","journal-title":"Int J Cancer"},{"key":"2026051916121986000_noag016-B132","doi-asserted-by":"publisher","first-page":"507","DOI":"10.1016\/j.ijrobp.2017.10.002","article-title":"Outcomes after reirradiation for recurrent pediatric intracranial ependymoma","volume":"100","author":"Tsang","year":"2018","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"2026051916121986000_noag016-B133","doi-asserted-by":"publisher","first-page":"905","DOI":"10.1007\/s00381-009-1067-4","article-title":"Treatment and outcome of children with relapsed ependymoma: a multi-institutional retrospective analysis","volume":"26","author":"Zacharoulis","year":"2010","journal-title":"Childs Nerv Syst"},{"key":"2026051916121986000_noag016-B134","doi-asserted-by":"crossref","first-page":"1815","DOI":"10.1016\/j.ejca.2009.03.018","article-title":"Relapsed intracranial ependymoma in children in the UK: patterns of relapse, survival and therapeutic outcome","volume":"45","author":"Messahel","year":"2009","journal-title":"Eur J Cancer"},{"key":"2026051916121986000_noag016-B135","doi-asserted-by":"publisher","first-page":"221","DOI":"10.1007\/s00381-004-1070-8","article-title":"Intracranial ependymomas in childhood: recurrence, reoperation, and outcome","volume":"21","author":"Vinchon","year":"2005","journal-title":"Childs Nerv Syst"},{"key":"2026051916121986000_noag016-B136","doi-asserted-by":"publisher","first-page":"1541","DOI":"10.1016\/j.ijrobp.2011.10.039","article-title":"Survival benefit for pediatric patients with recurrent ependymoma treated with reirradiation","volume":"83","author":"Bouffet","year":"2012","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"2026051916121986000_noag016-B137","doi-asserted-by":"publisher","first-page":"87","DOI":"10.1016\/j.ijrobp.2007.09.037","article-title":"A retrospective study of surgery and reirradiation for recurrent ependymoma","volume":"71","author":"Merchant","year":"2008","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"2026051916121986000_noag016-B138","doi-asserted-by":"publisher","first-page":"1293","DOI":"10.1007\/s00381-009-0883-x","article-title":"Salvage chemotherapy for metastatic and recurrent ependymoma of childhood","volume":"25","author":"Bouffet","year":"2009","journal-title":"Childs Nerv Syst"},{"key":"2026051916121986000_noag016-B139","doi-asserted-by":"publisher","first-page":"vdac053","DOI":"10.1093\/noajnl\/vdac053","article-title":"Phase II study of intravenous etoposide in patients with relapsed ependymoma (CNS 2001 04)","volume":"4","author":"Apps","year":"2022","journal-title":"\u00adNeurooncol Adv"},{"key":"2026051916121986000_noag016-B140","doi-asserted-by":"publisher","first-page":"131","DOI":"10.1007\/s11060-016-2155-4","article-title":"Single-agent erlotinib versus oral etoposide in patients with recurrent or refractory pediatric ependymoma: a randomized open-label study","volume":"129","author":"Jakacki","year":"2016","journal-title":"J Neurooncol"},{"key":"2026051916121986000_noag016-B141","doi-asserted-by":"publisher","first-page":"874","DOI":"10.1093\/neuonc\/noab066","article-title":"Pediatric ependymomas: destined to recur?","volume":"23","author":"Ritzmann","year":"2021","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B142","doi-asserted-by":"publisher","first-page":"887544","DOI":"10.3389\/fneur.2022.887544","article-title":"Toward improved diagnosis accuracy and treatment of children, adolescents, and young adults with ependymoma: the international SIOP ependymoma II protocol","volume":"13","author":"Leblond","year":"2022","journal-title":"Front Neurol"},{"key":"2026051916121986000_noag016-B143","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/noaf285","article-title":"Phase 3 randomized trial of post-irradiation chemotherapy in patients with newly diagnosed ependymoma, a report from the children\u2019s oncology group [published online ahead of print December 19, 2025]","author":"Smith","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B144","doi-asserted-by":"publisher","first-page":"1319","DOI":"10.1093\/neuonc\/noz069","article-title":"Molecular grouping and outcomes of young children with newly diagnosed ependymoma treated on the multi-institutional SJYC07 trial","volume":"21","author":"Upadhyaya","year":"2019","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B145","doi-asserted-by":"publisher","first-page":"165","DOI":"10.1038\/modpathol.3800999","article-title":"The prognostic value of histological grading of posterior fossa ependymomas in children: a children\u2019s oncology group study and a review of prognostic factors","volume":"21","author":"Tihan","year":"2008","journal-title":"Mod Pathol"},{"key":"2026051916121986000_noag016-B146","doi-asserted-by":"publisher","first-page":"v266","DOI":"10.1093\/neuonc\/noaf201.1059","article-title":"PTHP-02. The clinical value of histopathological and molecular metrics in pediatric ependymoma - data from the ACNS0831 trial","volume":"27","author":"Ellison","year":"2025","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B147","doi-asserted-by":"publisher","first-page":"445","DOI":"10.1093\/neuonc\/nox166","article-title":"EANO guidelines for the diagnosis and treatment of ependymal tumors","volume":"20","author":"Rud\u00e0","year":"2018","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B148","doi-asserted-by":"publisher","first-page":"52","DOI":"10.1016\/s0360-3016(01)02801-2","article-title":"Influence of tumor grade on time to progression after irradiation for localized ependymoma in children","volume":"53","author":"Merchant","year":"2002","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"2026051916121986000_noag016-B149","doi-asserted-by":"publisher","first-page":"100895","DOI":"10.1016\/j.neo.2023.100895","article-title":"Why haven\u2019t we solved intracranial pediatric ependymoma? Current questions and barriers to treatment advances","volume":"39","author":"Hwang","year":"2023","journal-title":"Neoplasia"},{"key":"2026051916121986000_noag016-B150","doi-asserted-by":"publisher","first-page":"1329","DOI":"10.1016\/j.cell.2020.04.047","article-title":"Metabolic regulation of the epigenome drives lethal infantile ependymoma","volume":"181","author":"Michealraj","year":"2020","journal-title":"Cell"},{"key":"2026051916121986000_noag016-B151","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1038\/nature25169","article-title":"Therapeutic targeting of ependymoma as informed by oncogenic enhancer profiling","volume":"553","author":"Mack","year":"2018","journal-title":"Nature"},{"key":"2026051916121986000_noag016-B152","doi-asserted-by":"publisher","first-page":"3750","DOI":"10.1158\/1078-0432.CCR-14-2650","article-title":"Spinal myxopapillary ependymomas demonstrate a warburg phenotype","volume":"21","author":"Mack","year":"2015","journal-title":"Clin Cancer Res"},{"key":"2026051916121986000_noag016-B153","doi-asserted-by":"publisher","first-page":"445","DOI":"10.1038\/nature13108","article-title":"Epigenomic alterations define lethal CIMP-positive ependymomas of infancy","volume":"506","author":"Mack","year":"2014","journal-title":"Nature"},{"key":"2026051916121986000_noag016-B154","doi-asserted-by":"publisher","first-page":"1544","DOI":"10.1038\/s41591-021-01404-8","article-title":"Locoregional infusion of HER2-specific CAR T cells in children and young adults with recurrent or refractory CNS tumors: an interim analysis","volume":"27","author":"Vitanza","year":"2021","journal-title":"Nat Med"},{"key":"2026051916121986000_noag016-B155","doi-asserted-by":"publisher","DOI":"10.1172\/JCI193332","article-title":"CAR-T-cells targeting the glycoprotein GD2 show potent anti-tumor efficacy in high-risk ependymoma models","volume":"136","author":"Tallon-Cobos","year":"2026","journal-title":"J Clin Invest"},{"key":"2026051916121986000_noag016-B156","doi-asserted-by":"publisher","first-page":"1854","DOI":"10.1093\/neuonc\/noad096","article-title":"Significant increase of high-risk chromosome 1q gain and 6q loss at recurrence in posterior fossa group A ependymoma: a multicenter study","volume":"25","author":"Donson","year":"2023","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B157","doi-asserted-by":"publisher","first-page":"786","DOI":"10.1093\/neuonc\/noac219","article-title":"Spatial transcriptomic analysis delineates epithelial and mesenchymal subpopulations and transition stages in childhood ependymoma","volume":"25","author":"Fu","year":"2023","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B158","doi-asserted-by":"publisher","first-page":"108023","DOI":"10.1016\/j.celrep.2020.108023","article-title":"Single-cell RNA sequencing of childhood ependymoma reveals neoplastic cell subpopulations that impact molecular classification and etiology","volume":"32","author":"Gillen","year":"2020","journal-title":"Cell Rep"},{"key":"2026051916121986000_noag016-B159","doi-asserted-by":"publisher","first-page":"44","DOI":"10.1016\/j.ccell.2020.06.004","article-title":"Single-cell RNA-seq reveals cellular hierarchies and impaired developmental trajectories in pediatric ependymoma","volume":"38","author":"Gojo","year":"2020","journal-title":"Cancer Cell"},{"key":"2026051916121986000_noag016-B160","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1038\/s41586-019-1158-7","article-title":"Childhood cerebellar tumours mirror conserved fetal transcriptional programs","volume":"572","author":"Vladoiu","year":"2019","journal-title":"Nature"},{"key":"2026051916121986000_noag016-B161","author":"Jeong"},{"key":"2026051916121986000_noag016-B162","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1093\/neuonc\/noae186","article-title":"Central nervous system tumors in adolescents and young adults: a society for Neuro-Oncology consensus review on diagnosis, management, and future directions","volume":"27","author":"Lim-Fat","year":"2024","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B163","doi-asserted-by":"publisher","first-page":"1286","DOI":"10.1093\/neuonc\/noad030","article-title":"Adult intracranial ependymoma-relevance of DNA methylation profiling for diagnosis, prognosis, and treatment","volume":"25","author":"Tr\u00e4ger","year":"2023","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B164","doi-asserted-by":"publisher","first-page":"1616","DOI":"10.1093\/neuonc\/noy118","article-title":"DNA methylation-based classification of ependymomas in adulthood: implications for diagnosis and treatment","volume":"20","author":"Witt","year":"2018","journal-title":"Neuro Oncol"},{"key":"2026051916121986000_noag016-B165","doi-asserted-by":"publisher","first-page":"767","DOI":"10.1007\/s00234-025-03553-w","article-title":"Recurrence patterns in pediatric intracranial ependymal neoplasm: a systematic imaging work-up","volume":"67","author":"Stock","year":"2025","journal-title":"Neuroradiology"}],"container-title":["Neuro-Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/neuro-oncology\/advance-article-pdf\/doi\/10.1093\/neuonc\/noag016\/66678234\/noag016.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/neuro-oncology\/article-pdf\/28\/5\/1085\/66678234\/noag016.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/neuro-oncology\/article-pdf\/28\/5\/1085\/66678234\/noag016.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T20:12:36Z","timestamp":1779221556000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/neuro-oncology\/article\/28\/5\/1085\/8450322"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1,31]]},"references-count":165,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2026,1,31]]},"published-print":{"date-parts":[[2026,5,1]]}},"URL":"https:\/\/doi.org\/10.1093\/neuonc\/noag016","relation":{},"ISSN":["1522-8517","1523-5866"],"issn-type":[{"value":"1522-8517","type":"print"},{"value":"1523-5866","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,5]]},"published":{"date-parts":[[2026,1,31]]}}}