{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T13:39:02Z","timestamp":1793108342631,"version":"4.5.0"},"reference-count":28,"publisher":"Springer Science and Business Media LLC","issue":"2","license":[{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,1,13]],"date-time":"2026-01-13T00:00:00Z","timestamp":1768262400000},"content-version":"vor","delay-in-days":12,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Deutsches Krebsforschungszentrum (DKFZ)","id":[{"id":"https:\/\/ror.org\/04cdgtt98","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurooncol"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Purpose<\/jats:title>\n                    <jats:p>H3 K27-altered diffuse midline glioma (DMG) is a molecularly defined, highly aggressive tumor entity since the 2016 revision of the World Health Organization (WHO) classification of tumors of the central nervous system (CNS). It has been most extensively characterized in children and adolescents and continues to pose significant challenges to treating physicians due to its growth along the midline structures of the brain and its ability to progress distantly leading to poor prognosis. Clinical and molecular determinants of distant progression and its impact on patient survival particularly in adults have not been thoroughly characterized.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This retrospective multicenter cohort study of 52 adult patients with DMG was analyzed for clinical and molecular predictors of distant tumor progression using gene panel sequencing and genome-wide DNA methylation arrays.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Distant progression including leptomeningeal disease occurred in 10 out of 52 adult patients (19%), seven of which had a primary spinal tumor location (70%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001) and almost 80% of patients with spinal DMG experienced distant progression. Across the entire cohort, distant progression was associated with worse progression free survival (PFS) (4.5 vs. 13 months;\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.037). However, we could not identify a genetic or methylation-based signature that was significantly correlated with the occurrence of distant progression.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Adult DMGs with spinal location are at a high risk of early distant progression. As there were no molecular determinants of distant progression from differential methylation, copy number alterations (CNA) or panel sequencing, adult patients with DMG should undergo routine craniospinal MRI, particularly in spinal tumor location.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s11060-025-05238-z","type":"journal-article","created":{"date-parts":[[2026,1,13]],"date-time":"2026-01-13T12:32:23Z","timestamp":1768307543000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Early distant progression in adult Histone-3 K27-altered diffuse midline gliomas"],"prefix":"10.1007","volume":"176","author":[{"given":"Niklas","family":"Grassl","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abigail K.","family":"Suwala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tobias","family":"Kessler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lukas","family":"Bunse","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iris","family":"Mildenberger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael O.","family":"Breckwoldt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miriam","family":"Ratliff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefanie","family":"Brehmer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christel","family":"Herold-Mende","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nima","family":"Etminan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfgang","family":"Wick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix","family":"Sahm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Platten","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7694-0988","authenticated-orcid":false,"given":"Katharina","family":"Sahm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,1,13]]},"reference":[{"key":"5238_CR1","doi-asserted-by":"publisher","first-page":"803","DOI":"10.1007\/s00401-016-1545-1","volume":"131","author":"DN Louis","year":"2016","unstructured":"Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW (2016) The 2016 world health organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 131:803\u2013820. https:\/\/doi.org\/10.1007\/s00401-016-1545-1","journal-title":"Acta Neuropathol"},{"key":"5238_CR2","doi-asserted-by":"publisher","first-page":"520","DOI":"10.1016\/j.ccell.2017.08.017","volume":"32","author":"A Mackay","year":"2017","unstructured":"Mackay A, Burford A, Carvalho D, Izquierdo E, Fazal-Salom J, Taylor KR, Bjerke L, Clarke M, Vinci M, Nandhabalan M, Temelso S, Popov S, Molinari V, Raman P, Waanders AJ, Han HJ, Gupta S, Marshall L, Zacharoulis S, Vaidya S, Mandeville HC, Bridges LR, Martin AJ, Al-Sarraj S, Chandler C, Ng HK, Li X, Mu K, Trabelsi S, Brahim DH, Kisljakov AN, Konovalov DM, Moore AS, Carcaboso AM, Sunol M, de Torres C, Cruz O, Mora J, Shats LI, Stavale JN, Bidinotto LT, Reis RM, Entz-Werle N, Farrell M, Cryan J, Crimmins D, Caird J, Pears J, Monje M, Debily MA, Castel D, Grill J, Hawkins C, Nikbakht H, Jabado N, Baker SJ, Pfister SM, Jones DTW, Fouladi M, von Bueren AO, Baudis M, Resnick A, Jones C (2017) Integrated molecular Meta-Analysis of 1,000 pediatric High-Grade and diffuse intrinsic Pontine glioma. Cancer Cell 32:520\u2013537e525. https:\/\/doi.org\/10.1016\/j.ccell.2017.08.017","journal-title":"Cancer Cell"},{"key":"5238_CR3","doi-asserted-by":"publisher","first-page":"123","DOI":"10.1093\/neuonc\/nox149","volume":"20","author":"M Karremann","year":"2018","unstructured":"Karremann M, Gielen GH, Hoffmann M, Wiese M, Colditz N, Warmuth-Metz M, Bison B, Claviez A, van Vuurden DG, von Bueren AO, Gessi M, K\u00fchnle I, Hans VH, Benesch M, Sturm D, Kortmann RD, Waha A, Pietsch T, Kramm CM (2018) Diffuse high-grade gliomas with H3 K27M mutations carry a dismal prognosis independent of tumor location. Neuro Oncol 20:123\u2013131. https:\/\/doi.org\/10.1093\/neuonc\/nox149","journal-title":"Neuro Oncol"},{"key":"5238_CR4","doi-asserted-by":"publisher","first-page":"602553","DOI":"10.3389\/fonc.2020.602553","volume":"10","author":"Y Wang","year":"2020","unstructured":"Wang Y, Feng LL, Ji PG, Liu JH, Guo SC, Zhai YL, Sankey EW, Wang Y, Xue YR, Wang N, Lou M, Xu M, Chao M, Gao GD, Qu Y, Gong L, Wang L (2020) Clinical features and molecular markers on diffuse midline gliomas with H3K27M mutations: A 43 cases retrospective cohort study. Front Oncol 10:602553. https:\/\/doi.org\/10.3389\/fonc.2020.602553","journal-title":"Front Oncol"},{"key":"5238_CR5","doi-asserted-by":"publisher","first-page":"331","DOI":"10.1126\/science.aao4750","volume":"360","author":"MG Filbin","year":"2018","unstructured":"Filbin MG, Tirosh I, Hovestadt V, Shaw ML, Escalante LE, Mathewson ND, Neftel C, Frank N, Pelton K, Hebert CM, Haberler C, Yizhak K, Gojo J, Egervari K, Mount C, van Galen P, Bonal DM, Nguyen QD, Beck A, Sinai C, Czech T, Dorfer C, Goumnerova L, Lavarino C, Carcaboso AM, Mora J, Mylvaganam R, Luo CC, Peyrl A, Popovi\u0107 M, Azizi A, Batchelor TT, Frosch MP, Martinez-Lage M, Kieran MW, Bandopadhayay P, Beroukhim R, Fritsch G, Getz G, Rozenblatt-Rosen O, Wucherpfennig KW, Louis DN, Monje M, Slavc I, Ligon KL, Golub TR, Regev A, Bernstein BE, Suv\u00e0 ML (2018) Developmental and oncogenic programs in H3K27M gliomas dissected by single-cell RNA-seq. Science 360:331\u2013335. https:\/\/doi.org\/10.1126\/science.aao4750","journal-title":"Science"},{"key":"5238_CR6","doi-asserted-by":"publisher","first-page":"1881","DOI":"10.1038\/s41588-022-01236-3","volume":"54","author":"I Liu","year":"2022","unstructured":"Liu I, Jiang L, Samuelsson ER, Marco Salas S, Beck A, Hack OA, Jeong D, Shaw ML, Englinger B, LaBelle J, Mire HM, Madlener S, Mayr L, Quezada MA, Trissal M, Panditharatna E, Ernst KJ, Vogelzang J, Gatesman TA, Halbert ME, Palova H, Pokorna P, Sterba J, Slaby O, Geyeregger R, Diaz A, Findlay IJ, Dun MD, Resnick A, Suv\u00e0 ML, Jones DTW, Agnihotri S, Svedlund J, Koschmann C, Haberler C, Czech T, Slavc I, Cotter JA, Ligon KL, Alexandrescu S, Yung WKA, Arrillaga-Romany I, Gojo J, Monje M, Nilsson M, Filbin MG (2022) The landscape of tumor cell States and Spatial organization in H3-K27M mutant diffuse midline glioma across age and location. Nat Genet 54:1881\u20131894. https:\/\/doi.org\/10.1038\/s41588-022-01236-3","journal-title":"Nat Genet"},{"key":"5238_CR7","doi-asserted-by":"publisher","first-page":"405","DOI":"10.1007\/s11060-022-04027-2","volume":"158","author":"HG Vuong","year":"2022","unstructured":"Vuong HG, Ngo TNM, Le HT, Dunn IF (2022) The prognostic significance of HIST1H3B\/C and H3F3A K27M mutations in diffuse midline gliomas is influenced by patient age. J Neurooncol 158:405\u2013412. https:\/\/doi.org\/10.1007\/s11060-022-04027-2","journal-title":"J Neurooncol"},{"key":"5238_CR8","doi-asserted-by":"publisher","first-page":"1479","DOI":"10.1093\/neuonc\/noae061","volume":"26","author":"A Ryba","year":"2024","unstructured":"Ryba A, \u00d6zdemir Z, Nissimov N, H\u00f6nikl L, Neidert N, Jakobs M, Kalasauskas D, Krigers A, Thom\u00e9 C, Freyschlag CF, Ringel F, Unterberg A, Dao Trong P, Beck J, Heiland DH, Meyer B, Vajkoczy P, Onken J, Stummer W, Suero Molina E, Gempt J, Westphal M, Sch\u00fcller U, Mohme M (2024) Insights from a multicenter study on adult H3 K27M-mutated glioma: surgical resection\u2019s limited influence on overall survival, ATRX as molecular prognosticator. Neuro Oncol 26:1479\u20131493. https:\/\/doi.org\/10.1093\/neuonc\/noae061","journal-title":"Neuro Oncol"},{"key":"5238_CR9","doi-asserted-by":"publisher","first-page":"225","DOI":"10.1007\/s11060-021-03890-9","volume":"155","author":"HG Vuong","year":"2021","unstructured":"Vuong HG, Le HT, Ngo TNM, Fung KM, Battiste JD, McNall-Knapp R, Dunn IF (2021) H3K27M-mutant diffuse midline gliomas should be further molecularly stratified: an integrated analysis of 669 patients. J Neurooncol 155:225\u2013234. https:\/\/doi.org\/10.1007\/s11060-021-03890-9","journal-title":"J Neurooncol"},{"key":"5238_CR10","doi-asserted-by":"publisher","first-page":"vdaa142","DOI":"10.1093\/noajnl\/vdaa142","volume":"2","author":"JD Schulte","year":"2020","unstructured":"Schulte JD, Buerki RA, Lapointe S, Molinaro AM, Zhang Y, Villanueva-Meyer JE, Perry A, Phillips JJ, Tihan T, Bollen AW, Pekmezci M, Butowski N, Oberheim Bush NA, Taylor JW, Chang SM, Theodosopoulos P, Aghi MK, Hervey-Jumper SL, Berger MS, Solomon DA, Clarke JL (2020) Clinical, radiologic, and genetic characteristics of histone H3 K27M-mutant diffuse midline gliomas in adults. Neurooncol Adv 2:vdaa142. https:\/\/doi.org\/10.1093\/noajnl\/vdaa142","journal-title":"Neurooncol Adv"},{"key":"5238_CR11","doi-asserted-by":"publisher","first-page":"40","DOI":"10.1007\/s00401-024-02800-3","volume":"148","author":"L Stegat","year":"2024","unstructured":"Stegat L, Eckhardt A, Gocke A, Neyazi S, Pohl L, Schmid S, Dottermusch M, Frank S, Pinnschmidt H, Herms J, Glatzel M, Snuderl M, Schweizer L, Thomas C, Neumann J, Dorostkar MM, Sch\u00fcller U, Wefers AK (2024) Integrated analyses reveal two molecularly and clinically distinct subtypes of H3 K27M-mutant diffuse midline gliomas with prognostic significance. Acta Neuropathol 148:40. https:\/\/doi.org\/10.1007\/s00401-024-02800-3","journal-title":"Acta Neuropathol"},{"key":"5238_CR12","doi-asserted-by":"publisher","first-page":"553","DOI":"10.1093\/neuonc\/noad161","volume":"26","author":"M Bruschi","year":"2024","unstructured":"Bruschi M, Midjek L, Ajlil Y, Vairy S, Lancien M, Ghermaoui S, Kergrohen T, Verreault M, Idbaih A, de Biagi CAOJ, Liu I, Filbin MG, Beccaria K, Blauwblomme T, Puget S, Tauziede-Espariat A, Varlet P, Dangouloff-Ros V, Boddaert N, Le Teuff G, Grill J, Montagnac G, Elkhatib N, Debily MA, Castel D (2024) Diffuse midline glioma invasion and metastasis rely on cell-autonomous signaling. Neuro Oncol 26:553\u2013568. https:\/\/doi.org\/10.1093\/neuonc\/noad161","journal-title":"Neuro Oncol"},{"key":"5238_CR13","doi-asserted-by":"publisher","first-page":"573","DOI":"10.1007\/s00401-014-1319-6","volume":"128","author":"P Buczkowicz","year":"2014","unstructured":"Buczkowicz P, Bartels U, Bouffet E, Becher O, Hawkins C (2014) Histopathological spectrum of paediatric diffuse intrinsic Pontine glioma: diagnostic and therapeutic implications. Acta Neuropathol 128:573\u2013581. https:\/\/doi.org\/10.1007\/s00401-014-1319-6","journal-title":"Acta Neuropathol"},{"key":"5238_CR14","doi-asserted-by":"publisher","first-page":"vdad068","DOI":"10.1093\/noajnl\/vdad068","volume":"5","author":"M Diaz","year":"2023","unstructured":"Diaz M, Rana S, Silva Correia CE, Reiner AS, Lin AL, Miller AM, Graham MS, Chudsky S, Bale TA, Rosenblum M, Karajannis MA, Pentsova E (2023) Leptomeningeal disease in histone-mutant gliomas. Neurooncol Adv 5:vdad068. https:\/\/doi.org\/10.1093\/noajnl\/vdad068","journal-title":"Neurooncol Adv"},{"key":"5238_CR15","doi-asserted-by":"publisher","first-page":"437","DOI":"10.1007\/s11060-024-04933-7","volume":"172","author":"S Zhong","year":"2025","unstructured":"Zhong S, Zuo J, Fu X, Wu C, Liu R, Huang Z, Li S (2025) Leptomeningeal dissemination in H3 K27M- mutant diffuse midline gliomas: clinical characteristics, risk factors, and prognostic insights. J Neurooncol 172:437\u2013445. https:\/\/doi.org\/10.1007\/s11060-024-04933-7","journal-title":"J Neurooncol"},{"key":"5238_CR16","doi-asserted-by":"publisher","first-page":"2262","DOI":"10.1093\/neuonc\/noad141","volume":"25","author":"M Monje","year":"2023","unstructured":"Monje M, Cooney T, Glod J, Huang J, Peer CJ, Faury D, Baxter P, Kramer K, Lenzen A, Robison NJ, Kilburn L, Vinitsky A, Figg WD, Jabado N, Fouladi M, Fangusaro J, Onar-Thomas A, Dunkel IJ, Warren KE (2023) Phase I trial of Panobinostat in children with diffuse intrinsic Pontine glioma: A report from the pediatric brain tumor consortium (PBTC-047). Neuro Oncol 25:2262\u20132272. https:\/\/doi.org\/10.1093\/neuonc\/noad141","journal-title":"Neuro Oncol"},{"key":"5238_CR17","doi-asserted-by":"publisher","first-page":"2586","DOI":"10.1038\/s41591-023-02555-6","volume":"29","author":"N Grassl","year":"2023","unstructured":"Grassl N, Poschke I, Lindner K, Bunse L, Mildenberger I, Boschert T, J\u00e4hne K, Green EW, H\u00fclsmeyer I, J\u00fcnger S, Kessler T, Suwala AK, Eisele P, Breckwoldt MO, Vajkoczy P, Grauer OM, Herrlinger U, Tonn JC, Denk M, Sahm F, Bendszus M, von Deimling A, Winkler F, Wick W, Platten M, Sahm K (2023) A H3K27M-targeted vaccine in adults with diffuse midline glioma. Nat Med 29:2586\u20132592. https:\/\/doi.org\/10.1038\/s41591-023-02555-6","journal-title":"Nat Med"},{"key":"5238_CR18","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1186\/s42466-023-00282-4","volume":"5","author":"N Grassl","year":"2023","unstructured":"Grassl N, Sahm K, S\u00fc\u00dfe H, Poschke I, Bunse L, Bunse T, Boschert T, Mildenberger I, Rupp AK, Ewinger MP, Lanz LM, Denk M, Tabatabai G, Ronellenfitsch MW, Herrlinger U, Glas M, Krex D, Vajkoczy P, Wick A, Harting I, Sahm F, von Deimling A, Bendszus M, Wick W, Platten M (2023) INTERCEPT H3: a multicenter phase I peptide vaccine trial for the treatment of H3-mutated diffuse midline gliomas. Neurol Res Pract 5:55. https:\/\/doi.org\/10.1186\/s42466-023-00282-4","journal-title":"Neurol Res Pract"},{"key":"5238_CR19","doi-asserted-by":"publisher","first-page":"934","DOI":"10.1038\/s41586-022-04489-4","volume":"603","author":"RG Majzner","year":"2022","unstructured":"Majzner RG, Ramakrishna S, Yeom KW, Patel S, Chinnasamy H, Schultz LM, Richards RM, Jiang L, Barsan V, Mancusi R, Geraghty AC, Good Z, Mochizuki AY, Gillespie SM, Toland AMS, Mahdi J, Reschke A, Nie EH, Chau IJ, Rotiroti MC, Mount CW, Baggott C, Mavroukakis S, Egeler E, Moon J, Erickson C, Green S, Kunicki M, Fujimoto M, Ehlinger Z, Reynolds W, Kurra S, Warren KE, Prabhu S, Vogel H, Rasmussen L, Cornell TT, Partap S, Fisher PG, Campen CJ, Filbin MG, Grant G, Sahaf B, Davis KL, Feldman SA, Mackall CL, Monje M (2022) GD2-CAR T cell therapy for H3K27M-mutated diffuse midline gliomas. Nature 603:934\u2013941. https:\/\/doi.org\/10.1038\/s41586-022-04489-4","journal-title":"Nature"},{"key":"5238_CR20","doi-asserted-by":"publisher","first-page":"708","DOI":"10.1038\/s41586-024-08171-9","volume":"637","author":"M Monje","year":"2025","unstructured":"Monje M, Mahdi J, Majzner R, Yeom KW, Schultz LM, Richards RM, Barsan V, Song KW, Kamens J, Baggott C, Kunicki M, Rietberg SP, Lim AS, Reschke A, Mavroukakis S, Egeler E, Moon J, Patel S, Chinnasamy H, Erickson C, Jacobs A, Duh AK, Tunuguntla R, Klysz DD, Fowler C, Green S, Beebe B, Carr C, Fujimoto M, Brown AK, Petersen AG, McIntyre C, Siddiqui A, Lepori-Bui N, Villar K, Pham K, Bove R, Musa E, Reynolds WD, Kuo A, Prabhu S, Rasmussen L, Cornell TT, Partap S, Fisher PG, Campen CJ, Grant G, Prolo L, Ye X, Sahaf B, Davis KL, Feldman SA, Ramakrishna S, Mackall C (2025) Intravenous and intracranial GD2-CAR T cells for H3K27M(+) diffuse midline gliomas. Nature 637:708\u2013715. https:\/\/doi.org\/10.1038\/s41586-024-08171-9","journal-title":"Nature"},{"key":"5238_CR21","doi-asserted-by":"publisher","first-page":"S173","DOI":"10.1093\/neuonc\/noae031","volume":"26","author":"I Arrillaga-Romany","year":"2024","unstructured":"Arrillaga-Romany I, Lassman A, McGovern SL, Mueller S, Nabors B, van den Bent M, Vogelbaum MA, Allen JE, Melemed AS, Tarapore RS, Wen PY, Cloughesy T (2024) ACTION: a randomized phase 3 study of ONC201 (dordaviprone) in patients with newly diagnosed H3 K27M-mutant diffuse glioma. Neuro Oncol 26:S173\u2013s181. https:\/\/doi.org\/10.1093\/neuonc\/noae031","journal-title":"Neuro Oncol"},{"key":"5238_CR22","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1007\/s11060-019-03271-3","volume":"145","author":"AS Chi","year":"2019","unstructured":"Chi AS, Tarapore RS, Hall MD, Shonka N, Gardner S, Umemura Y, Sumrall A, Khatib Z, Mueller S, Kline C, Zaky W, Khatua S, Weathers SP, Odia Y, Niazi TN, Daghistani D, Cherrick I, Korones D, Karajannis MA, Kong XT, Minturn J, Waanders A, Arillaga-Romany I, Batchelor T, Wen PY, Merdinger K, Schalop L, Stogniew M, Allen JE, Oster W, Mehta MP (2019) Pediatric and adult H3 K27M-mutant diffuse midline glioma treated with the selective DRD2 antagonist ONC201. J Neurooncol 145:97\u2013105. https:\/\/doi.org\/10.1007\/s11060-019-03271-3","journal-title":"J Neurooncol"},{"key":"5238_CR23","doi-asserted-by":"publisher","first-page":"1542","DOI":"10.1200\/jco.23.01134","volume":"42","author":"I Arrillaga-Romany","year":"2024","unstructured":"Arrillaga-Romany I, Gardner SL, Odia Y, Aguilera D, Allen JE, Batchelor T, Butowski N, Chen C, Cloughesy T, Cluster A, de Groot J, Dixit KS, Graber JJ, Haggiagi AM, Harrison RA, Kheradpour A, Kilburn LB, Kurz SC, Lu G, MacDonald TJ, Mehta M, Melemed AS, Nghiemphu PL, Ramage SC, Shonka N, Sumrall A, Tarapore RS, Taylor L, Umemura Y, Wen PY (2024) ONC201 (Dordaviprone) in recurrent H3 K27M-Mutant diffuse midline glioma. J Clin Oncol 42:1542\u20131552. https:\/\/doi.org\/10.1200\/jco.23.01134","journal-title":"J Clin Oncol"},{"key":"5238_CR24","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, Brat DJ, Cree IA, Figarella-Branger D, Hawkins C, Ng HK, Pfister SM, Reifenberger G, Soffietti R, von Deimling A, Ellison DW (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":"5238_CR25","doi-asserted-by":"publisher","unstructured":"Capper D, Jones DTW, Sill M, Hovestadt V, Schrimpf D, Sturm D, Koelsche C, Sahm F, Chavez L, Reuss DE, Kratz A, Wefers AK, Huang K, Pajtler KW, Schweizer L, Stichel D, Olar A, Engel NW, Lindenberg K, Harter PN, Braczynski AK, Plate KH, Dohmen H, Garvalov BK, Coras R, H\u00f6lsken A, Hewer E, Bewerunge-Hudler M, Schick M, Fischer R, Beschorner R, Schittenhelm J, Staszewski O, Wani K, Varlet P, Pages M, Temming P, Lohmann D, Selt F, Witt H, Milde T, Witt O, Aronica E, Giangaspero F, Rushing E, Scheurlen W, Geisenberger C, Rodriguez FJ, Becker A, Preusser M, Haberler C, Bjerkvig R, Cryan J, Farrell M, Deckert M, Hench J, Frank S, Serrano J, Kannan K, Tsirigos A, Br\u00fcck W, Hofer S, Brehmer S, Seiz-Rosenhagen M, H\u00e4nggi D, Hans V, Rozsnoki S, Hansford JR, Kohlhof P, Kristensen BW, Lechner M, Lopes B, Mawrin C, Ketter R, Kulozik A, Khatib Z, Heppner F, Koch A, Jouvet A, Keohane C, M\u00fchleisen H, Mueller W, Pohl U, Prinz M, Benner A, Zapatka M, Gottardo NG, Driever PH, Kramm CM, M\u00fcller HL, Rutkowski S, von Hoff K, Fr\u00fchwald MC, Gnekow A, Fleischhack G, Tippelt S, Calaminus G, Monoranu CM, Perry A, Jones C, Jacques TS, Radlwimmer B, Gessi M, Pietsch T, Schramm J, Schackert G, Westphal M, Reifenberger G, Wesseling P, Weller M, Collins VP, Bl\u00fcmcke I, Bendszus M, Debus J, Huang A, Jabado N, Northcott PA, Paulus W, Gajjar A, Robinson GW, Taylor MD, Jaunmuktane Z, Ryzhova M, Platten M, Unterberg A, Wick W, Karajannis MA, Mittelbronn M, Acker T, Hartmann C, Aldape K, Sch\u00fcller U, Buslei R, Lichter P, Kool M, Herold-Mende C, Ellison DW, Hasselblatt M, Snuderl M, Brandner S, Korshunov A, von Deimling A, Pfister SM (2018) DNA methylation-based classification of central nervous system tumours. Nature 555: 469\u2013474 https:\/\/doi.org\/10.1038\/nature26000","DOI":"10.1038\/nature26000"},{"key":"5238_CR26","doi-asserted-by":"publisher","first-page":"307","DOI":"10.1007\/s13311-016-0507-6","volume":"14","author":"BM Ellingson","year":"2017","unstructured":"Ellingson BM, Wen PY, Cloughesy TF (2017) Modified criteria for radiographic response assessment in glioblastoma clinical trials. Neurotherapeutics 14:307\u2013320. https:\/\/doi.org\/10.1007\/s13311-016-0507-6","journal-title":"Neurotherapeutics"},{"key":"5238_CR27","doi-asserted-by":"publisher","first-page":"523","DOI":"10.1001\/archneurol.2011.2910","volume":"69","author":"F Sahm","year":"2012","unstructured":"Sahm F, Capper D, Jeibmann A, Habel A, Paulus W, Troost D, von Deimling A (2012) Addressing diffuse glioma as a systemic brain disease with single-cell analysis. Arch Neurol 69:523\u2013526. https:\/\/doi.org\/10.1001\/archneurol.2011.2910","journal-title":"Arch Neurol"},{"key":"5238_CR28","doi-asserted-by":"publisher","first-page":"e330","DOI":"10.1016\/s1470-2045(20)30166-2","volume":"21","author":"TM Cooney","year":"2020","unstructured":"Cooney TM, Cohen KJ, Guimaraes CV, Dhall G, Leach J, Massimino M, Erbetta A, Chiapparini L, Malbari F, Kramer K, Pollack IF, Baxter P, Laughlin S, Patay Z, Young Poussaint T, Warren KE (2020) Response assessment in diffuse intrinsic Pontine glioma: recommendations from the response assessment in pediatric Neuro-Oncology (RAPNO) working group. Lancet Oncol 21:e330\u2013e336. https:\/\/doi.org\/10.1016\/s1470-2045(20)30166-2","journal-title":"Lancet Oncol"}],"container-title":["Journal of Neuro-Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s11060-025-05238-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s11060-025-05238-z","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s11060-025-05238-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T12:52:14Z","timestamp":1793105534000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s11060-025-05238-z"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1]]},"references-count":28,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026,1]]}},"alternative-id":["5238"],"URL":"https:\/\/doi.org\/10.1007\/s11060-025-05238-z","relation":{},"ISSN":["0167-594X","1573-7373"],"issn-type":[{"value":"0167-594X","type":"print"},{"value":"1573-7373","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1]]},"assertion":[{"value":"5 September 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 October 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 January 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 October 2026","order":5,"name":"change_date","label":"Change Date","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Update","order":6,"name":"change_type","label":"Change Type","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The original version of this article has been revised: The (missing) supplementary information has been added online.","order":7,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"M.P. is founder of Tcelltech and inventor of associated intellectual property related to glioma-reactive T cell receptors (patent no. WO2022200456A1). M.P., K.S. and L.B. are inventors of associated intellectual property related to H3K27M vaccines (patent nos. EP3118217A1 and US20180155403A1). The remaining authors declare no competing interests.","order":1,"name":"Ethics","label":"Competing interests","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"166"}}