{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T18:49:56Z","timestamp":1792090196251,"version":"4.4.0"},"reference-count":67,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T00:00:00Z","timestamp":1790035200000},"content-version":"vor","delay-in-days":21,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurooncol"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Purpose<\/jats:title>\n                    <jats:p>To review the clinical, molecular, and imaging landscape of posterior fossa (PF) ependymoma and outline current management principles guiding surgical and adjuvant treatment decisions.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We performed a narrative review of the contemporary literature on molecular classification, imaging characteristics, and treatment outcomes of PF ependymoma, focusing on distinctions between posterior fossa group A (PFA) and group B (PFB) tumors.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>PFA and PFB ependymomas are molecularly and clinically distinct. PFA tumors predominate in younger children, follow a more aggressive course, and show loss of H3K27me3, EZHIP overexpression, Moreover, in PFA tumors 1q gain and\/or 6q loss defines a very high-risk group with increased recurrence and metastasis. PFB tumors arise more often in older patients, generally retain H3K27me3, harbor numerous arm-level chromosomal gains\/losses, and carry more favorable outcomes. On MRI, PFA tumors more frequently show larger volume, hydrocephalus, lateral extension, skull base involvement, and lower apparent diffusion coefficient values. Gross total resection remains the most important prognostic factor and should be pursued whenever safe. Focal postoperative radiotherapy may be standard for localized disease, while chemotherapy has only a limited adjunctive role. Recurrence, especially among PFA tumors, is managed with early detection, repeat resection, and selective re-irradiation; 1q gain and 6q loss are enriched at relapse.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>PF ependymoma comprises two biologically and clinically distinct entities. Maximal safe resection and focal radiotherapy remain the backbone of treatment, but integrating molecular subgrouping, imaging biomarkers, and modern radiotherapeutic techniques into surgical and adjuvant planning offers the clearest path to improved outcomes, particularly for high-risk PFA tumors.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s11060-026-05808-9","type":"journal-article","created":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T17:15:32Z","timestamp":1790097332000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Posterior fossa ependymoma: a comprehensive review of molecular classification, management guidelines, and clinical outcomes (Part I of ependymomas across compartments)"],"prefix":"10.1007","volume":"179","author":[{"given":"George W.","family":"Koutsouras","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francisco","family":"Rivera","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony M.","family":"Price","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Derek S.","family":"Tsang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Esbenshade","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vijay","family":"Ramaswamy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter B.","family":"Dirks","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael C.","family":"Dewan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,9,22]]},"reference":[{"key":"5808_CR1","doi-asserted-by":"publisher","first-page":"vi1","DOI":"10.1093\/neuonc\/noae145","volume":"26","author":"M Price","year":"2024","unstructured":"Price M, Ballard C, Benedetti J et al (2024) CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2017\u20132021. Neuro Oncol 26:vi1\u2013vi85. https:\/\/doi.org\/10.1093\/neuonc\/noae145","journal-title":"Neuro Oncol"},{"key":"5808_CR2","doi-asserted-by":"publisher","first-page":"v1","DOI":"10.1093\/neuonc\/noac202","volume":"24","author":"QT Ostrom","year":"2022","unstructured":"Ostrom QT, Price M, Neff C et al (2022) CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2015\u20132019. Neuro Oncol 24:v1\u2013v95. https:\/\/doi.org\/10.1093\/neuonc\/noac202","journal-title":"Neuro Oncol"},{"key":"5808_CR3","doi-asserted-by":"publisher","first-page":"1228","DOI":"10.1016\/j.jocn.2012.02.006","volume":"19","author":"K Vaidya","year":"2012","unstructured":"Vaidya K, Smee R, Williams JR (2012) Prognostic factors and treatment options for paediatric ependymomas. J Clin Neurosci 19:1228\u20131235. https:\/\/doi.org\/10.1016\/j.jocn.2012.02.006","journal-title":"J Clin Neurosci"},{"key":"5808_CR4","doi-asserted-by":"publisher","first-page":"3182","DOI":"10.1200\/JCO.2009.27.3359","volume":"28","author":"A Korshunov","year":"2010","unstructured":"Korshunov A, Witt H, Hielscher T et al (2010) Molecular staging of intracranial ependymoma in children and adults. J Clin Oncol 28:3182\u20133190. https:\/\/doi.org\/10.1200\/JCO.2009.27.3359","journal-title":"J Clin Oncol"},{"key":"5808_CR5","doi-asserted-by":"publisher","first-page":"143","DOI":"10.1016\/j.ccr.2011.07.007","volume":"20","author":"H Witt","year":"2011","unstructured":"Witt H, Mack SC, Ryzhova M et al (2011) Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma. Cancer Cell 20:143\u2013157. https:\/\/doi.org\/10.1016\/j.ccr.2011.07.007","journal-title":"Cancer Cell"},{"key":"5808_CR6","doi-asserted-by":"publisher","first-page":"728","DOI":"10.1016\/j.ccell.2015.04.002","volume":"27","author":"KW Pajtler","year":"2015","unstructured":"Pajtler KW, Witt H, Sill M et al (2015) Molecular Classification of Ependymal Tumors across All CNS Compartments, Histopathological Grades, and Age Groups. Cancer Cell 27:728\u2013743. https:\/\/doi.org\/10.1016\/j.ccell.2015.04.002","journal-title":"Cancer Cell"},{"key":"5808_CR7","doi-asserted-by":"publisher","first-page":"863","DOI":"10.1111\/bpa.12866","volume":"30","author":"DW Ellison","year":"2020","unstructured":"Ellison DW, Aldape KD, Capper D et al (2020) cIMPACT-NOW update 7: advancing the molecular classification of ependymal tumors. Brain Pathol 30:863\u2013866. https:\/\/doi.org\/10.1111\/bpa.12866","journal-title":"Brain Pathol"},{"key":"5808_CR8","doi-asserted-by":"publisher","first-page":"134","DOI":"10.1186\/s40478-018-0630-1","volume":"6","author":"K Fukuoka","year":"2018","unstructured":"Fukuoka K, Kanemura Y, Shofuda T et al (2018) Significance of molecular classification of ependymomas: C11orf95-RELA fusion-negative supratentorial ependymomas are a heterogeneous group of tumors. Acta Neuropathol Commun 6:134. https:\/\/doi.org\/10.1186\/s40478-018-0630-1","journal-title":"Acta Neuropathol Commun"},{"key":"5808_CR9","doi-asserted-by":"publisher","first-page":"1231","DOI":"10.1093\/neuonc\/noab106","volume":"23","author":"DN Louis","year":"2021","unstructured":"Louis DN, Perry A, Wesseling P et al (2021) The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol 23:1231\u20131251. https:\/\/doi.org\/10.1093\/neuonc\/noab106","journal-title":"Neuro Oncol"},{"key":"5808_CR10","doi-asserted-by":"publisher","first-page":"133","DOI":"10.1016\/j.ccr.2011.08.003","volume":"20","author":"TC Archer","year":"2011","unstructured":"Archer TC, Pomeroy SL (2011) Posterior fossa ependymomas: a tale of two subtypes. Cancer Cell 20:133\u2013134. https:\/\/doi.org\/10.1016\/j.ccr.2011.08.003","journal-title":"Cancer Cell"},{"key":"5808_CR11","doi-asserted-by":"publisher","first-page":"2146","DOI":"10.1038\/s41467-019-09981-6","volume":"10","author":"SU Jain","year":"2019","unstructured":"Jain SU, Do TJ, Lund PJ et al (2019) PFA ependymoma-associated protein EZHIP inhibits PRC2 activity through a H3 K27M-like mechanism. Nat Commun 10:2146. https:\/\/doi.org\/10.1038\/s41467-019-09981-6","journal-title":"Nat Commun"},{"key":"5808_CR12","doi-asserted-by":"publisher","first-page":"878","DOI":"10.1093\/neuonc\/noz058","volume":"21","author":"J-M H\u00fcbner","year":"2019","unstructured":"H\u00fcbner J-M, M\u00fcller T, Papageorgiou DN et al (2019) EZHIP\/CXorf67 mimics K27M mutated oncohistones and functions as an intrinsic inhibitor of PRC2 function in aggressive posterior fossa ependymoma. Neuro Oncol 21:878\u2013889. https:\/\/doi.org\/10.1093\/neuonc\/noz058","journal-title":"Neuro Oncol"},{"key":"5808_CR13","doi-asserted-by":"publisher","first-page":"705","DOI":"10.1007\/s00401-017-1752-4","volume":"134","author":"P Panwalkar","year":"2017","unstructured":"Panwalkar P, Clark J, Ramaswamy V et al (2017) Immunohistochemical analysis of H3K27me3 demonstrates global reduction in group-A childhood posterior fossa ependymoma and is a powerful predictor of outcome. Acta Neuropathol 134:705\u2013714. https:\/\/doi.org\/10.1007\/s00401-017-1752-4","journal-title":"Acta Neuropathol"},{"key":"5808_CR14","doi-asserted-by":"publisher","first-page":"88","DOI":"10.1186\/s40478-016-0349-9","volume":"4","author":"A Araki","year":"2016","unstructured":"Araki A, Chocholous M, Gojo J et al (2016) Chromosome 1q gain and tenascin-C expression are candidate markers to define different risk groups in pediatric posterior fossa ependymoma. Acta Neuropathol Commun 4:88. https:\/\/doi.org\/10.1186\/s40478-016-0349-9","journal-title":"Acta Neuropathol Commun"},{"key":"5808_CR15","doi-asserted-by":"publisher","first-page":"1867","DOI":"10.1002\/cncr.31995","volume":"125","author":"M Zapotocky","year":"2019","unstructured":"Zapotocky M, Beera K, Adamski J et al (2019) Survival and functional outcomes of molecularly defined childhood posterior fossa ependymoma: Cure at a cost. Cancer 125:1867\u20131876. https:\/\/doi.org\/10.1002\/cncr.31995","journal-title":"Cancer"},{"key":"5808_CR16","doi-asserted-by":"publisher","first-page":"1360","DOI":"10.1093\/neuonc\/noab034","volume":"23","author":"LV Baroni","year":"2021","unstructured":"Baroni LV, Sundaresan L, Heled A et al (2021) Ultra high-risk PFA ependymoma is characterized by loss of chromosome 6q. Neuro Oncol 23:1360\u20131370. https:\/\/doi.org\/10.1093\/neuonc\/noab034","journal-title":"Neuro Oncol"},{"key":"5808_CR17","doi-asserted-by":"publisher","first-page":"366ra161","DOI":"10.1126\/scitranslmed.aah6904","volume":"8","author":"J Bayliss","year":"2016","unstructured":"Bayliss J, Mukherjee P, Lu C et al (2016) Lowered H3K27me3 and DNA hypomethylation define poorly prognostic pediatric posterior fossa ependymomas. Sci Transl Med 8:366ra161. https:\/\/doi.org\/10.1126\/scitranslmed.aah6904","journal-title":"Sci Transl Med"},{"key":"5808_CR18","doi-asserted-by":"publisher","first-page":"101459","DOI":"10.1016\/j.neuchi.2023.101459","volume":"69","author":"RG Boukaka","year":"2023","unstructured":"Boukaka RG, Szathmari A, Di Rocco F et al (2023) Posterior fossa ependymoma in children: A long-term single-center experience. Neurochirurgie 69:101459. https:\/\/doi.org\/10.1016\/j.neuchi.2023.101459","journal-title":"Neurochirurgie"},{"key":"5808_CR19","doi-asserted-by":"publisher","first-page":"23","DOI":"10.1016\/s0887-8994(97)00162-8","volume":"18","author":"AM Comi","year":"1998","unstructured":"Comi AM, Backstrom JW, Burger PC, Duffner PK (1998) Clinical and neuroradiologic findings in infants with intracranial ependymomas. Pediatr Oncol Group Pediatr Neurol 18:23\u201329. https:\/\/doi.org\/10.1016\/s0887-8994(97)00162-8","journal-title":"Pediatr Oncol Group Pediatr Neurol"},{"key":"5808_CR20","doi-asserted-by":"publisher","first-page":"651","DOI":"10.1227\/neu.0000000000002923","volume":"95","author":"B Wang","year":"2024","unstructured":"Wang B, Yan M, Han B et al (2024) Impact of Molecular Subgroups on Prognosis and Survival Outcomes in Posterior Fossa Ependymomas: A Retrospective Study of 412 Cases. Neurosurgery 95:651\u2013659. https:\/\/doi.org\/10.1227\/neu.0000000000002923","journal-title":"Neurosurgery"},{"key":"5808_CR21","doi-asserted-by":"publisher","first-page":"1181211","DOI":"10.3389\/fped.2023.1181211","volume":"11","author":"W Mu","year":"2023","unstructured":"Mu W, Dahmoush H (2023) Classification and neuroimaging of ependymal tumors. Front Pediatr 11:1181211. https:\/\/doi.org\/10.3389\/fped.2023.1181211","journal-title":"Front Pediatr"},{"key":"5808_CR22","doi-asserted-by":"publisher","first-page":"1203","DOI":"10.1007\/s00381-009-0878-7","volume":"25","author":"EL Yuh","year":"2009","unstructured":"Yuh EL, Barkovich AJ, Gupta N (2009) Imaging of ependymomas: MRI and CT. Childs Nerv Syst 25:1203\u20131213. https:\/\/doi.org\/10.1007\/s00381-009-0878-7","journal-title":"Childs Nerv Syst"},{"key":"5808_CR23","doi-asserted-by":"publisher","first-page":"1765","DOI":"10.1007\/s00381-010-1251-6","volume":"26","author":"JM U-King-Im","year":"2010","unstructured":"U-King-Im JM, Taylor MD, Raybaud C (2010) Posterior fossa ependymomas: new radiological classification with surgical correlation. Childs Nerv Syst 26:1765\u20131772. https:\/\/doi.org\/10.1007\/s00381-010-1251-6","journal-title":"Childs Nerv Syst"},{"key":"5808_CR24","doi-asserted-by":"publisher","first-page":"1534","DOI":"10.1007\/s00330-023-10182-5","volume":"34","author":"T Leclerc","year":"2024","unstructured":"Leclerc T, Levy R, Tauzi\u00e8de-Espariat A et al (2024) Imaging features to distinguish posterior fossa ependymoma subgroups. Eur Radiol 34:1534\u20131544. https:\/\/doi.org\/10.1007\/s00330-023-10182-5","journal-title":"Eur Radiol"},{"key":"5808_CR25","doi-asserted-by":"publisher","first-page":"287","DOI":"10.1007\/s11060-005-6753-9","volume":"75","author":"TE Merchant","year":"2005","unstructured":"Merchant TE, Fouladi M (2005) Ependymoma: new therapeutic approaches including radiation and chemotherapy. J Neurooncol 75:287\u2013299. https:\/\/doi.org\/10.1007\/s11060-005-6753-9","journal-title":"J Neurooncol"},{"key":"5808_CR26","doi-asserted-by":"publisher","first-page":"629","DOI":"10.3171\/jns.2005.102.4.0629","volume":"102","author":"L Rogers","year":"2005","unstructured":"Rogers L, Pueschel J, Spetzler R et al (2005) Is gross-total resection sufficient treatment for posterior fossa ependymomas? J Neurosurg 102:629\u2013636. https:\/\/doi.org\/10.3171\/jns.2005.102.4.0629","journal-title":"J Neurosurg"},{"key":"5808_CR27","doi-asserted-by":"publisher","first-page":"985","DOI":"10.1007\/s11912-022-01260-w","volume":"24","author":"R Rud\u00e0","year":"2022","unstructured":"Rud\u00e0 R, Bruno F, Pellerino A, Soffietti R (2022) Ependymoma: Evaluation and Management Updates. Curr Oncol Rep 24:985\u2013993. https:\/\/doi.org\/10.1007\/s11912-022-01260-w","journal-title":"Curr Oncol Rep"},{"key":"5808_CR28","doi-asserted-by":"publisher","first-page":"132","DOI":"10.3171\/2022.10.PEDS22154","volume":"31","author":"EY Montgomery","year":"2023","unstructured":"Montgomery EY, Thirunavu V, Pagadala M et al (2023) Recurrent pediatric infratentorial ependymomas: a systematic review and meta-analysis on outcomes and molecular classification. J Neurosurg Pediatr 31:132\u2013142. https:\/\/doi.org\/10.3171\/2022.10.PEDS22154","journal-title":"J Neurosurg Pediatr"},{"key":"5808_CR29","doi-asserted-by":"publisher","first-page":"974","DOI":"10.1200\/JCO.18.01765","volume":"37","author":"TE Merchant","year":"2019","unstructured":"Merchant TE, Bendel AE, Sabin ND et al (2019) Conformal Radiation Therapy for Pediatric Ependymoma, Chemotherapy for Incompletely Resected Ependymoma, and Observation for Completely Resected, Supratentorial Ependymoma. J Clin Oncol 37:974\u2013983. https:\/\/doi.org\/10.1200\/JCO.18.01765","journal-title":"J Clin Oncol"},{"key":"5808_CR30","doi-asserted-by":"publisher","first-page":"404","DOI":"10.3171\/2023.5.PEDS22350","volume":"32","author":"MDSda Costa","year":"2023","unstructured":"Costa MDSda, Torres Soares C, Dastoli PA et al (2023) Survival analysis and prognostic factors in posterior fossa ependymomas in children and adolescents. J Neurosurg Pediatr 32:404\u2013412. https:\/\/doi.org\/10.3171\/2023.5.PEDS22350","journal-title":"J Neurosurg Pediatr"},{"key":"5808_CR31","doi-asserted-by":"publisher","first-page":"1286","DOI":"10.1093\/neuonc\/noaf285","volume":"28","author":"AA Smith","year":"2026","unstructured":"Smith AA, Onar-Thomas A, Lensing S et al (2026) Phase 3 randomized trial of postirradiation chemotherapy in patients with newly diagnosed ependymoma: A report from the Children\u2019s Oncology Group. Neuro Oncol 28:1286\u20131297. https:\/\/doi.org\/10.1093\/neuonc\/noaf285","journal-title":"Neuro Oncol"},{"key":"5808_CR32","doi-asserted-by":"publisher","first-page":"695","DOI":"10.3171\/jns.1998.88.4.0695","volume":"88","author":"PL Robertson","year":"1998","unstructured":"Robertson PL, Zeltzer PM, Boyett JM et al (1998) Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children\u2019s Cancer Group. J Neurosurg 88:695\u2013703. https:\/\/doi.org\/10.3171\/jns.1998.88.4.0695","journal-title":"J Neurosurg"},{"key":"5808_CR33","doi-asserted-by":"publisher","first-page":"246","DOI":"10.3171\/2011.6.PEDS1142","volume":"8","author":"M Massimino","year":"2011","unstructured":"Massimino M, Solero CL, Garr\u00e8 ML et al (2011) Second-look surgery for ependymoma: the Italian experience. J Neurosurg Pediatr 8:246\u2013250. https:\/\/doi.org\/10.3171\/2011.6.PEDS1142","journal-title":"J Neurosurg Pediatr"},{"key":"5808_CR34","doi-asserted-by":"publisher","first-page":"856","DOI":"10.1097\/00006123-199704000-00042","volume":"40","author":"NK Foreman","year":"1997","unstructured":"Foreman NK, Love S, Gill SS, Coakham HB (1997) Second-look surgery for incompletely resected fourth ventricle ependymomas: technical case report. Neurosurgery 40:856\u2013860 discussion 860. https:\/\/doi.org\/10.1097\/00006123-199704000-00042","journal-title":"Neurosurgery"},{"key":"5808_CR35","doi-asserted-by":"publisher","first-page":"367","DOI":"10.3171\/2023.11.PEDS23364","volume":"33","author":"AK Malhotra","year":"2024","unstructured":"Malhotra AK, Nobre L, Ibrahim GM et al (2024) Complications following resection of primary and recurrent pediatric posterior fossa ependymoma. J Neurosurg Pediatr 33:367\u2013373. https:\/\/doi.org\/10.3171\/2023.11.PEDS23364","journal-title":"J Neurosurg Pediatr"},{"key":"5808_CR36","doi-asserted-by":"publisher","first-page":"391","DOI":"10.1007\/s11060-009-9866-8","volume":"94","author":"EB Morris","year":"2009","unstructured":"Morris EB, Li C, Khan RB et al (2009) Evolution of neurological impairment in pediatric infratentorial ependymoma patients. J Neurooncol 94:391\u2013398. https:\/\/doi.org\/10.1007\/s11060-009-9866-8","journal-title":"J Neurooncol"},{"key":"5808_CR37","doi-asserted-by":"publisher","first-page":"98","DOI":"10.3171\/2020.1.PEDS19541","volume":"26","author":"MA LoPresti","year":"2020","unstructured":"LoPresti MA, Nguyen J, Lam SK (2020) Pinning in pediatric neurosurgery: the modified rubber stopper technique. J Neurosurg Pediatr 26:98\u2013103. https:\/\/doi.org\/10.3171\/2020.1.PEDS19541","journal-title":"J Neurosurg Pediatr"},{"key":"5808_CR38","doi-asserted-by":"publisher","first-page":"2697","DOI":"10.1007\/s00381-024-06492-8","volume":"40","author":"K Klein Gunnewiek","year":"2024","unstructured":"Klein Gunnewiek K, van Baarsen KM, Graus EHM et al (2024) Navigated intraoperative ultrasound in pediatric brain tumors. Childs Nerv Syst 40:2697\u20132705. https:\/\/doi.org\/10.1007\/s00381-024-06492-8","journal-title":"Childs Nerv Syst"},{"key":"5808_CR39","doi-asserted-by":"publisher","first-page":"170","DOI":"10.1007\/s00381-026-07281-1","volume":"42","author":"AK Aftahy","year":"2026","unstructured":"Aftahy AK, Giordano M, Koehnen N et al (2026) Intraoperative MRI in pediatric brain tumor surgery: an 18-year single-center experience. Childs Nerv Syst 42:170. https:\/\/doi.org\/10.1007\/s00381-026-07281-1","journal-title":"Childs Nerv Syst"},{"key":"5808_CR40","doi-asserted-by":"publisher","first-page":"2079","DOI":"10.1007\/s00381-023-05917-0","volume":"39","author":"C Hedrich","year":"2023","unstructured":"Hedrich C, Gojo J, Azizi A et al (2023) Placement of EVD in pediatric posterior fossa tumors: safe and efficient or old-fashioned? The Vienna experience. Childs Nerv Syst 39:2079\u20132086. https:\/\/doi.org\/10.1007\/s00381-023-05917-0","journal-title":"Childs Nerv Syst"},{"key":"5808_CR41","doi-asserted-by":"publisher","first-page":"461","DOI":"10.1093\/ons\/opz194","volume":"18","author":"PJ Morone","year":"2020","unstructured":"Morone PJ, Dewan MC, Zuckerman SL et al (2020) Craniometrics and Ventricular Access: A Review of Kocher\u2019s, Kaufman\u2019s, Paine\u2019s, Menovksy\u2019s, Tubbs\u2019, Keen\u2019s, Frazier\u2019s, Dandy\u2019s, and Sanchez\u2019s Points. Oper Neurosurg 18:461\u2013469. https:\/\/doi.org\/10.1093\/ons\/opz194","journal-title":"Oper Neurosurg"},{"key":"5808_CR42","doi-asserted-by":"publisher","first-page":"3156","DOI":"10.1200\/JCO.2004.11.142","volume":"22","author":"TE Merchant","year":"2004","unstructured":"Merchant TE, Mulhern RK, Krasin MJ et al (2004) Preliminary results from a phase II trial of conformal radiation therapy and evaluation of radiation-related CNS effects for pediatric patients with localized ependymoma. J Clin Oncol 22:3156\u20133162. https:\/\/doi.org\/10.1200\/JCO.2004.11.142","journal-title":"J Clin Oncol"},{"key":"5808_CR43","doi-asserted-by":"publisher","first-page":"2570","DOI":"10.1002\/cncr.30623","volume":"123","author":"M Sato","year":"2017","unstructured":"Sato M, Gunther JR, Mahajan A et al (2017) Progression-free survival of children with localized ependymoma treated with intensity-modulated radiation therapy or proton-beam radiation therapy. Cancer 123:2570\u20132578. https:\/\/doi.org\/10.1002\/cncr.30623","journal-title":"Cancer"},{"key":"5808_CR44","doi-asserted-by":"publisher","first-page":"258","DOI":"10.1016\/s1470-2045(08)70342-5","volume":"10","author":"TE Merchant","year":"2009","unstructured":"Merchant TE, Li C, Xiong X et al (2009) Conformal radiotherapy after surgery for paediatric ependymoma: a prospective study. Lancet Oncol 10:258\u2013266. https:\/\/doi.org\/10.1016\/s1470-2045(08)70342-5","journal-title":"Lancet Oncol"},{"key":"5808_CR45","doi-asserted-by":"publisher","first-page":"547","DOI":"10.1016\/j.ijrobp.2014.06.043","volume":"90","author":"TE Merchant","year":"2014","unstructured":"Merchant TE, Sharma S, Xiong X et al (2014) Effect of Cerebellum Radiation Dosimetry on Cognitive Outcomes in Children With Infratentorial Ependymoma. Int J Radiat Oncol Biol Phys 90:547\u2013553. https:\/\/doi.org\/10.1016\/j.ijrobp.2014.06.043","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"5808_CR46","doi-asserted-by":"publisher","first-page":"936","DOI":"10.1093\/neuonc\/noac012","volume":"24","author":"TA Ritzmann","year":"2022","unstructured":"Ritzmann TA, Chapman RJ, Kilday J-P et al (2022) SIOP Ependymoma I: Final results, long-term follow-up, and molecular analysis of the trial cohort-A BIOMECA Consortium Study. Neuro Oncol 24:936\u2013948. https:\/\/doi.org\/10.1093\/neuonc\/noac012","journal-title":"Neuro Oncol"},{"key":"5808_CR47","doi-asserted-by":"publisher","first-page":"815","DOI":"10.1016\/j.ijrobp.2021.01.027","volume":"110","author":"DJ Indelicato","year":"2021","unstructured":"Indelicato DJ, Ioakeim-Ioannidou M, Bradley JA et al (2021) Proton Therapy for Pediatric Ependymoma: Mature Results From a Bicentric Study. Int J Radiat Oncol Biol Phys 110:815\u2013820. https:\/\/doi.org\/10.1016\/j.ijrobp.2021.01.027","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"5808_CR48","doi-asserted-by":"publisher","first-page":"387","DOI":"10.1016\/j.ijrobp.2016.06.2446","volume":"96","author":"DJ Indelicato","year":"2016","unstructured":"Indelicato DJ, Merchant T, Laperriere N et al (2016) Consensus Report From the Stockholm Pediatric Proton Therapy Conference. Int J Radiat Oncol Biol Phys 96:387\u2013392. https:\/\/doi.org\/10.1016\/j.ijrobp.2016.06.2446","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"5808_CR49","doi-asserted-by":"publisher","first-page":"359","DOI":"10.1016\/j.ijrobp.2026.03.027","volume":"125","author":"JD Parkes","year":"2026","unstructured":"Parkes JD, Esiashvili N, Tsang DS et al (2026) Proton Beam Radiation Therapy for All Children and Adolescents Globally: Is This a Realistic and Cost-Effective Goal? Int J Radiation Oncology*Biology*Physics 125:359\u2013365. https:\/\/doi.org\/10.1016\/j.ijrobp.2026.03.027","journal-title":"Int J Radiation Oncology*Biology*Physics"},{"key":"5808_CR50","doi-asserted-by":"publisher","first-page":"e32155","DOI":"10.1002\/pbc.32155","volume":"73","author":"N Esiashvili","year":"2026","unstructured":"Esiashvili N, Indelicato DJ, Mahajan A et al (2026) Selection of the Most Appropriate Radiation Treatment Modality for Children\u2019s Cancers: A Paediatric Radiation Oncology Society (PROS) Position Paper on the Ethical Utilization of Proton Therapy. Pediatr Blood Cancer 73:e32155. https:\/\/doi.org\/10.1002\/pbc.32155","journal-title":"Pediatr Blood Cancer"},{"key":"5808_CR51","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1016\/j.ijrobp.2018.01.013","volume":"101","author":"D Haas-Kogan","year":"2018","unstructured":"Haas-Kogan D, Indelicato D, Paganetti H et al (2018) National Cancer Institute Workshop on Proton Therapy for Children: Considerations Regarding Brainstem Injury. Int J Radiation Oncology*Biology*Physics 101:152\u2013168. https:\/\/doi.org\/10.1016\/j.ijrobp.2018.01.013","journal-title":"Int J Radiation Oncology*Biology*Physics"},{"key":"5808_CR52","doi-asserted-by":"publisher","first-page":"156","DOI":"10.1093\/neuonc\/noaa139","volume":"23","author":"BE Patteson","year":"2021","unstructured":"Patteson BE, Baliga S, Bajaj BVM et al (2021) Clinical outcomes in a large pediatric cohort of patients with ependymoma treated with proton radiotherapy. Neuro Oncol 23:156\u2013166. https:\/\/doi.org\/10.1093\/neuonc\/noaa139","journal-title":"Neuro Oncol"},{"key":"5808_CR53","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1016\/j.ijrobp.2018.01.013","volume":"101","author":"D Haas-Kogan","year":"2018","unstructured":"Haas-Kogan D, Indelicato D, Paganetti H et al (2018) National Cancer Institute Workshop on Proton Therapy for Children: Considerations Regarding Brainstem Injury. Int J Radiat Oncol Biol Phys 101:152\u2013168. https:\/\/doi.org\/10.1016\/j.ijrobp.2018.01.013","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"5808_CR54","doi-asserted-by":"publisher","first-page":"e27697","DOI":"10.1002\/pbc.27697","volume":"66","author":"BJ Ager","year":"2019","unstructured":"Ager BJ, Christensen MT, Burt LM, Poppe MM (2019) The value of high-dose radiotherapy in intracranial ependymoma. Pediatr Blood Cancer 66:e27697. https:\/\/doi.org\/10.1002\/pbc.27697","journal-title":"Pediatr Blood Cancer"},{"key":"5808_CR55","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1007\/s00401-016-1643-0","volume":"133","author":"KW Pajtler","year":"2017","unstructured":"Pajtler KW, Mack SC, Ramaswamy V et al (2017) The current consensus on the clinical management of intracranial ependymoma and its distinct molecular variants. Acta Neuropathol 133:5\u201312. https:\/\/doi.org\/10.1007\/s00401-016-1643-0","journal-title":"Acta Neuropathol"},{"key":"5808_CR56","doi-asserted-by":"publisher","first-page":"227","DOI":"10.1007\/s00401-018-1888-x","volume":"136","author":"FMG Cavalli","year":"2018","unstructured":"Cavalli FMG, H\u00fcbner J-M, Sharma T et al (2018) Heterogeneity within the PF-EPN-B ependymoma subgroup. Acta Neuropathol 136:227\u2013237. https:\/\/doi.org\/10.1007\/s00401-018-1888-x","journal-title":"Acta Neuropathol"},{"key":"5808_CR57","doi-asserted-by":"publisher","first-page":"1416","DOI":"10.1002\/cam4.713","volume":"5","author":"C Wetmore","year":"2016","unstructured":"Wetmore C, Daryani VM, Billups CA et al (2016) Phase II evaluation of sunitinib in the treatment of recurrent or refractory high-grade glioma or ependymoma in children: a children\u2019s Oncology Group Study ACNS1021. Cancer Med 5:1416\u20131424. https:\/\/doi.org\/10.1002\/cam4.713","journal-title":"Cancer Med"},{"key":"5808_CR58","doi-asserted-by":"publisher","first-page":"1544","DOI":"10.1158\/1078-0432.CCR-23-3156","volume":"30","author":"AM Griesinger","year":"2024","unstructured":"Griesinger AM, Calzadilla AJ, Grimaldo E et al (2024) Development of Chromosome 1q+ Specific Treatment for Highest Risk Pediatric Posterior Fossa Ependymoma. Clin Cancer Res 30:1544\u20131554. https:\/\/doi.org\/10.1158\/1078-0432.CCR-23-3156","journal-title":"Clin Cancer Res"},{"key":"5808_CR59","doi-asserted-by":"publisher","first-page":"2468","DOI":"10.1200\/JCO.2015.65.7825","volume":"34","author":"V Ramaswamy","year":"2016","unstructured":"Ramaswamy V, Hielscher T, Mack SC et al (2016) Therapeutic Impact of Cytoreductive Surgery and Irradiation of Posterior Fossa Ependymoma in the Molecular Era: A Retrospective Multicohort Analysis. J Clin Oncol 34:2468\u20132477. https:\/\/doi.org\/10.1200\/JCO.2015.65.7825","journal-title":"J Clin Oncol"},{"key":"5808_CR60","doi-asserted-by":"publisher","first-page":"573","DOI":"10.1007\/s11060-023-04258-x","volume":"161","author":"AK Malhotra","year":"2023","unstructured":"Malhotra AK, Nobre LF, Ibrahim GM et al (2023) Outcomes following management of relapsed pediatric posterior fossa ependymoma in the molecular era. J Neurooncol 161:573\u2013582. https:\/\/doi.org\/10.1007\/s11060-023-04258-x","journal-title":"J Neurooncol"},{"key":"5808_CR61","doi-asserted-by":"publisher","first-page":"231","DOI":"10.1002\/pbc.21276","volume":"50","author":"S Zacharoulis","year":"2008","unstructured":"Zacharoulis S, Ji L, Pollack IF et al (2008) Metastatic ependymoma: a multi-institutional retrospective analysis of prognostic factors. Pediatr Blood Cancer 50:231\u2013235. https:\/\/doi.org\/10.1002\/pbc.21276","journal-title":"Pediatr Blood Cancer"},{"key":"5808_CR62","doi-asserted-by":"publisher","first-page":"767","DOI":"10.1007\/s00234-025-03553-w","volume":"67","author":"A Stock","year":"2025","unstructured":"Stock A, Krumma J, Fleischhack G et al (2025) Recurrence patterns in pediatric intracranial ependymal neoplasm: a systematic imaging work-up. Neuroradiology 67:767\u2013781. https:\/\/doi.org\/10.1007\/s00234-025-03553-w","journal-title":"Neuroradiology"},{"key":"5808_CR63","doi-asserted-by":"publisher","first-page":"1854","DOI":"10.1093\/neuonc\/noad096","volume":"25","author":"AM Donson","year":"2023","unstructured":"Donson AM, Bertrand KC, Riemondy KA et al (2023) Significant increase of high-risk chromosome 1q gain and 6q loss at recurrence in posterior fossa group A ependymoma: A multicenter study. Neuro Oncol 25:1854\u20131867. https:\/\/doi.org\/10.1093\/neuonc\/noad096","journal-title":"Neuro Oncol"},{"key":"5808_CR64","doi-asserted-by":"publisher","first-page":"vdab158","DOI":"10.1093\/noajnl\/vdab158","volume":"3","author":"DY Mak","year":"2021","unstructured":"Mak DY, Laperriere N, Ramaswamy V et al (2021) Reevaluating surgery and re-irradiation for locally recurrent pediatric ependymoma-a multi-institutional study. Neurooncol Adv 3:vdab158. https:\/\/doi.org\/10.1093\/noajnl\/vdab158","journal-title":"Neurooncol Adv"},{"key":"5808_CR65","doi-asserted-by":"publisher","first-page":"1293","DOI":"10.1007\/s00381-009-0883-x","volume":"25","author":"E Bouffet","year":"2009","unstructured":"Bouffet E, Capra M, Bartels U (2009) Salvage chemotherapy for metastatic and recurrent ependymoma of childhood. Childs Nerv Syst 25:1293\u20131301. https:\/\/doi.org\/10.1007\/s00381-009-0883-x","journal-title":"Childs Nerv Syst"},{"key":"5808_CR66","doi-asserted-by":"publisher","first-page":"547","DOI":"10.1093\/neuonc\/noy191","volume":"21","author":"DS Tsang","year":"2019","unstructured":"Tsang DS, Murray L, Ramaswamy V et al (2019) Craniospinal irradiation as part of re-irradiation for children with recurrent intracranial ependymoma. Neuro Oncol 21:547\u2013557. https:\/\/doi.org\/10.1093\/neuonc\/noy191","journal-title":"Neuro Oncol"},{"key":"5808_CR67","doi-asserted-by":"publisher","first-page":"879","DOI":"10.1186\/s40064-016-2562-1","volume":"5","author":"MJ Lob\u00f3n","year":"2016","unstructured":"Lob\u00f3n MJ, Bautista F, Riet F et al (2016) Re-irradiation of recurrent pediatric ependymoma: modalities and outcomes: a twenty-year survey. Springerplus 5:879. https:\/\/doi.org\/10.1186\/s40064-016-2562-1","journal-title":"Springerplus"}],"container-title":["Journal of Neuro-Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s11060-026-05808-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s11060-026-05808-9","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s11060-026-05808-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T18:01:39Z","timestamp":1792087299000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s11060-026-05808-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9]]},"references-count":67,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026,9]]}},"alternative-id":["5808"],"URL":"https:\/\/doi.org\/10.1007\/s11060-026-05808-9","relation":{},"ISSN":["0167-594X","1573-7373"],"issn-type":[{"value":"0167-594X","type":"print"},{"value":"1573-7373","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9]]},"assertion":[{"value":"9 August 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 September 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 September 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"This is an observational and review study. Ethics board approval is not required.","order":1,"name":"Ethics","label":"Ethics and consent to participate","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","label":"Competing interests","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"96"}}