{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T19:41:01Z","timestamp":1787168461700,"version":"build-2736575974"},"reference-count":13,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T00:00:00Z","timestamp":1779321600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T00:00:00Z","timestamp":1779321600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100020027","name":"Dr. Rolf M. Schwiete Stiftung","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100020027","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology Appl Neurobio"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Tumours historically classified as extraventricular neurocytoma (EVN), initially in comparison with central neurocytoma, are defined by location and morphology; however, emerging molecular data demonstrate that this term is outdated. We report 14 previously unpublished tumours with a robust EVN DNA methylation profile, independent of original histopathologic diagnosis or anatomic location. Integrated molecular analyses identified\n                    <jats:italic>FGFR1<\/jats:italic>\n                    alterations, particularly\n                    <jats:italic>FGFR1::TACC1<\/jats:italic>\n                    fusions, as the predominant molecular driver of this tumour class. A smaller number of tumours harboured\n                    <jats:italic>FGFR1<\/jats:italic>\n                    hotspot mutations (p.N546K and p.K656E). Among these, two cases additionally showed\n                    <jats:italic>NF1<\/jats:italic>\n                    variants, and one tumour carried a\n                    <jats:italic>PIK3CA<\/jats:italic>\n                    mutation (p.H1047L). In addition, a single case exhibited an\n                    <jats:italic>NTRK2<\/jats:italic>\n                    fusion. Histologically, tumours exhibited neurocytic differentiation with neuropil islands and a minor glial component, while immunohistochemistry showed frequent OLIG2 expression, contrasting with the typical immunophenotype of central neurocytoma. Notably, tumours with an EVN DNA methylation profile were identified in both extraventricular and intraventricular locations, demonstrating that anatomy\u2010based definitions are inadequate for this tumour type. Clinical follow\u2010up (median 33.2\u2009months) showed predominantly lower grade behaviour, with occasional recurrence. Collectively, these findings support the view that tumours previously labelled as EVN do not represent a direct counterpart of central neurocytoma but instead constitute a molecularly and epigenetically distinct glioneuronal tumour type. We propose the designation glioneuronal tumour with neurocytic differentiation (GNTN) to reflect its biological identity and to align the terminology with contemporary molecular neuropathology, and recommend DNA methylation profiling as an essential diagnostic criterion.\n                  <\/jats:p>","DOI":"10.1111\/nan.70080","type":"journal-article","created":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T08:20:58Z","timestamp":1779351658000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Glioneuronal Tumour With Neurocytic Differentiation (GNTN): a Molecularly Defined Tumour Type Formerly Referred to as Extraventricular Neurocytoma"],"prefix":"10.1111","volume":"52","author":[{"given":"Alice","family":"M\u00e9tais","sequence":"first","affiliation":[{"name":"Department of Neuropathology, GHU Paris \u2010 Psychiatry and Neuroscience Sainte\u2010Anne Hospital  Paris France"},{"name":"Translational Research in Neuroscience Lab, Institut Imagine Universit\u00e9 Paris Cit\u00e9  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Myriam","family":"Edjlali","sequence":"additional","affiliation":[{"name":"Multimodal Biomedical Imaging Laboratory (BioMaps), Fr\u00e9d\u00e9ric Joliot Hospital Department Paris\u2010Saclay University, CEA, CNRS, Inserm  Orsay France"},{"name":"Department of Radiology APHP  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Farah","family":"Sassi","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, GHU Paris \u2010 Psychiatry and Neuroscience Sainte\u2010Anne Hospital  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Chotard","sequence":"additional","affiliation":[{"name":"Department of Pathology University Hospital of Bordeaux, Pellegrin Hospital  Bordeaux France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Uro\u2010Coste","sequence":"additional","affiliation":[{"name":"Department of Pathology Toulouse University Hospital  Toulouse France"},{"name":"INSERM U1037 Cancer Research Center of Toulouse (CRCT)  Toulouse France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benoit","family":"Lhermitte","sequence":"additional","affiliation":[{"name":"Pathology Department University Hospitals of Strasbourg  Strasbourg France"},{"name":"Laboratory Bioimaging and Pathologies UMR CNRS 7021, OnKO3T Team, Faculty of Pharmacy  Illkirch France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romain","family":"Appay","sequence":"additional","affiliation":[{"name":"Neuro\u2010Pathology Department, AP\u2010HM University Hospital La Timone  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Pinto","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Institute of Pathology University Hospital Heidelberg  Heidelberg Germany"},{"name":"Clinical Cooperation Unit Neuropathology, German Consortium for Translational Cancer Research (DKTK) German Cancer Research Center (DKFZ)  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6642-7774","authenticated-orcid":false,"given":"Christian","family":"Thomas","sequence":"additional","affiliation":[{"name":"Institute of Neuropathology University Hospital M\u00fcnster  M\u00fcnster Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9887-7668","authenticated-orcid":false,"given":"Luca","family":"Bertero","sequence":"additional","affiliation":[{"name":"Pathology Unit, Department of Medical Sciences University of Turin  Turin Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miriam","family":"Ratliff","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University Hospital Mannheim, Medical Faculty Mannheim University of Heidelberg  Mannheim Germany"},{"name":"Clinical Cooperation Unit Neurooncology, German Consortium for Translational Cancer Research (DKTK) German Cancer Research Center (DKFZ)  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4050-1531","authenticated-orcid":false,"given":"Sandro M.","family":"Krieg","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Heidelberg University Hospital  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"von Deimling","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Institute of Pathology University Hospital Heidelberg  Heidelberg Germany"},{"name":"Clinical Cooperation Unit Neuropathology, German Consortium for Translational Cancer Research (DKTK) German Cancer Research Center (DKFZ)  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5441-1962","authenticated-orcid":false,"given":"Felix","family":"Sahm","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Institute of Pathology University Hospital Heidelberg  Heidelberg Germany"},{"name":"Clinical Cooperation Unit Neuropathology, German Consortium for Translational Cancer Research (DKTK) German Cancer Research Center (DKFZ)  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascale","family":"Varlet","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, GHU Paris \u2010 Psychiatry and Neuroscience Sainte\u2010Anne Hospital  Paris France"},{"name":"Institut de Psychiatrie et Neurosciences de Paris (IPNP), UMR S1266, INSERM, IMA\u2010BRAIN  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3237-6021","authenticated-orcid":false,"given":"Philipp","family":"Sievers","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Institute of Pathology University Hospital Heidelberg  Heidelberg Germany"},{"name":"Clinical Cooperation Unit Neuropathology, German Consortium for Translational Cancer Research (DKTK) German Cancer Research Center (DKFZ)  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,5,21]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401\u2010018\u20101882\u20103"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccell.2025.11.002"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40478\u2010020\u201001027\u2010z"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.13223"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature26000"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401\u2010015\u20101519\u20108"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10014\u2010023\u201000469\u20102"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/jscr\/rjad699"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40478\u2010020\u201000898\u20106"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41467\u2010025\u201061820\u2010z"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/s43018\u2010025\u201000962\u2010x"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401\u2010025\u201002894\u20103"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/s12276\u2010024\u201001204\u20103"}],"container-title":["Neuropathology and Applied Neurobiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/nan.70080","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/nan.70080","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/nan.70080","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T03:54:01Z","timestamp":1782186841000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/nan.70080"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,21]]},"references-count":13,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026,6]]}},"alternative-id":["10.1111\/nan.70080"],"URL":"https:\/\/doi.org\/10.1111\/nan.70080","archive":["Portico"],"relation":{},"ISSN":["0305-1846","1365-2990"],"issn-type":[{"value":"0305-1846","type":"print"},{"value":"1365-2990","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5,21]]},"assertion":[{"value":"2026-04-20","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-04-30","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-05-21","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70080"}}