{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T05:17:02Z","timestamp":1788239822174,"version":"build-2803163510"},"reference-count":23,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:p>\n                    Myxoid glioneuronal tumor (MGNT), a rare neuroepithelial neoplasm newly recognized in the 2021 World Health Organization (WHO) Classification of Tumors of the Central Nervous System (5th edition), is associated with platelet\u2010derived growth factor receptor \u03b1 (\n                    <jats:italic>PDGFRA)<\/jats:italic>\n                    gene alterations. While MGNT typically exhibits indolent histopathological features and a favorable clinical course, rare cases of intraventricular dissemination and leptomeningeal metastasis have been documented. We report a case of a 10-year-old female who presented with headache and was found to have an MGNT in the septum pellucidum. Gross total resection was achieved, and histopathological examination confirmed the diagnosis. Targeted next-generation sequencing (NGS) revealed concurrent pathogenic variants in\n                    <jats:italic>PDGFRA<\/jats:italic>\n                    and fibroblast growth factor receptor 3 (\n                    <jats:italic>FGFR3).<\/jats:italic>\n                    To contextualize this finding, we performed a systematic review of published MGNT literature to synthesize its clinicopathological, immunohistochemical, and molecular features. Our analysis indicates that integrating immunohistochemistry with molecular profiling\u2014especially NGS\u2014is essential for precise diagnosis and risk stratification. This case broadens the known molecular spectrum of MGNT and implies\n                    <jats:italic>FGFR3<\/jats:italic>\n                    as a potential therapeutic target, warranting further investigation of FGFR-directed inhibitors in selected patients.\n                  <\/jats:p>","DOI":"10.3389\/fonc.2026.1903894","type":"journal-article","created":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T04:27:18Z","timestamp":1788236838000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Myxoid glioneuronal tumor of the septum pellucidum with concurrent dual PDGFRA and FGFR3 gene mutations: a case report and literature review"],"prefix":"10.3389","volume":"16","author":[{"given":"Meng","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Pathology, Children\u2019s Hospital of Hebei Province, Hebei Provincial Clinical Research Center for Child Health and Disease","place":["Shijiazhuang, Hebei, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lingyan","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Children\u2019s Hospital of Hebei Province, Hebei Provincial Clinical Research Center for Child Health and Disease","place":["Shijiazhuang, Hebei, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lili","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Children\u2019s Hospital of Hebei Province, Hebei Provincial Clinical Research Center for Child Health and Disease","place":["Shijiazhuang, Hebei, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jianwei","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Children\u2019s Hospital of Hebei Province, Hebei Provincial Clinical Research Center for Child Health and Disease","place":["Shijiazhuang, Hebei, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yonghui","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Children\u2019s Hospital of Hebei Province, Hebei Provincial Clinical Research Center for Child Health and Disease","place":["Shijiazhuang, Hebei, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pathology, Children\u2019s Hospital of Hebei Province, Hebei Provincial Clinical Research Center for Child Health and Disease","place":["Shijiazhuang, Hebei, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,9,1]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.13060","article-title":"The 2021 WHO classification of tumors of the central nervous system: An update on pediatric low\u2010grade gliomas and glioneuronal tumors","volume":"32","author":"Bale","year":"2022","journal-title":"Brain Pathol"},{"key":"B2","doi-asserted-by":"publisher","first-page":"2","DOI":"10.1007\/s00381-024-06660-w","article-title":"Myxoid glioneuronal tumor of the septum pellucidum in pediatric patients: A case report and comprehensive review of the literature","volume":"41","author":"Oktay","year":"2025","journal-title":"Childs Nerv Syst"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.13008","article-title":"Myxoid glioneuronal tumor, PDGFRA p.K385L\u2010mutant, arising in midbrain tectum with multifocal CSF dissemination","volume":"32","author":"Kleinschmidt\u2010DeMasters","year":"2022","journal-title":"Brain Pathol"},{"key":"B4","doi-asserted-by":"publisher","first-page":"7204","DOI":"10.1038\/s41598-024-57859-5","article-title":"PDGFRA K385 mutants in myxoid glioneuronal tumors promote receptor dimerization and oncogenic signaling","volume":"14","author":"De Villenfagne","year":"2024","journal-title":"Sci Rep"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12797","article-title":"Myxoid glioneuronal tumor, PDGFRA p.K385\u2010mutant: Clinical, radiologic, and histopathologic features","volume":"30","author":"Lucas","year":"2020","journal-title":"Brain Pathol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.13079","article-title":"Temporal lobe myxoid glioneuronal tumor, PDGFRA p.K385L\u2010mutant with DNA methylation confirmation","volume":"32","author":"Gilani","year":"2022","journal-title":"Brain Pathol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1093\/bjrcr\/uaae014","article-title":"One case of myxoid glioneuronal tumour in the left frontal lobe","volume":"10","author":"Chu","year":"2024","journal-title":"BJRCase Rep"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.32074\/1591-951X-N1071","article-title":"Myxoid glioneuronal tumor with PDGFRA p.K385 mutation in the periventricular white matter: A rare case and literature insights","volume":"117","author":"Salzano","year":"2025","journal-title":"Pathologica"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1259\/bjrcr.20200139","article-title":"Myxoid glioneuronal tumour \u2013 Report of three cases of a new tumour in a typical location and review of literature","volume":"7","author":"Narvaez","year":"2021","journal-title":"BJRcase Rep"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.2176\/jns-nmc.2025-0028","article-title":"Myxoid glioneuronal tumours with PDGFRA p.K385 mutations causing hydrocephalus and successfully treated with neuroendoscopic surgery: A case report and literature review","volume":"12","author":"Wakisaka","year":"2025","journal-title":"NMC Case Rep J"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2023.1263556","article-title":"Memory deficit following resection of an intraventricular myxoid glioneuronal tumor impinging on the bilateral fornix: A case report","volume":"13","author":"Stasenko","year":"2023","journal-title":"Front Oncol"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1093\/braincomms\/fcae156","article-title":"Enrichment of oligodendrocyte precursor phenotypes in subsets of low-grade glioneuronal tumours","volume":"6","author":"Duan","year":"2024","journal-title":"Brain Commun"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1016\/j.neo.2023.100885","article-title":"Myxoid glioneuronal tumor: Histopathologic, neuroradiologic, and molecular features in a single center series","volume":"37","author":"Caporalini","year":"2023","journal-title":"Neoplasia"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-018-1883-2","article-title":"Myxoid glioneuronal tumor of the septum pellucidum and lateral ventricle is defined by a recurrent PDGFRA p.K385 mutation and DNT-like methylation profile","volume":"136","author":"Solomon","year":"2018","journal-title":"Acta Neuropathol (Berl)"},{"key":"B15","doi-asserted-by":"publisher","first-page":"699","DOI":"10.1007\/s00234-023-03253-3","article-title":"Imaging of supratentorial intraventricular masses in children: A pictorial review\u2014part 2","volume":"66","author":"Goncalves","year":"2024","journal-title":"Neuroradiology"},{"key":"B16","doi-asserted-by":"publisher","first-page":"139","DOI":"10.1186\/s13046-025-03386-8","article-title":"Targeted inhibition of PDGFRA with avapritinib, markedly enhances lenvatinib efficacy in hepatocellular carcinoma in vitro and in vivo: Clinical implications","volume":"44","author":"Zhao","year":"2025","journal-title":"J Exp Clin Cancer Res"},{"key":"B17","doi-asserted-by":"publisher","first-page":"589","DOI":"10.1002\/dvdy.564","article-title":"Characterizing the role of Pdgfra in calvarial development","volume":"252","author":"Umar","year":"2023","journal-title":"Dev Dyn"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.30699\/ijp.14.2.148","article-title":"Role of FGFR3 in urothelial carcinoma","volume":"14","year":"2019","journal-title":"Iran J Pathol"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.18632\/oncotarget.13139","article-title":"FGFR3, as a receptor tyrosine kinase, is associated with differentiated biological functions and improved survival of glioma patients","volume":"7","author":"Wang","year":"2016","journal-title":"Oncotarget"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1093\/nop\/npaa075","article-title":"FGFR3 overexpression is a useful detection tool for FGFR3 fusions and sequence variations in glioma","volume":"8","author":"Schittenhelm","year":"2021","journal-title":"Neuro-Oncol Pract"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-24-4708","article-title":"Spatial multiomics defines a shared tumor infiltrative signature at the resection margin in high-grade gliomas","volume":"85","author":"Pai","year":"2025","journal-title":"Cancer Res"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1016\/j.wneu.2019.05.006","article-title":"Multifocal infratentorial pilocytic astrocytoma in an adult patient","volume":"128","author":"Silva","year":"2019","journal-title":"World Neurosurg"},{"key":"B23","doi-asserted-by":"publisher","first-page":"e13917","DOI":"10.7759\/cureus.13917","article-title":"A case of dysembryoplastic neuroepithelial tumor in an adolescent male","volume":"13","author":"Yibirin","year":"2021","journal-title":"Cureus"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1903894\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T04:27:19Z","timestamp":1788236839000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1903894\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,1]]},"references-count":23,"alternative-id":["10.3389\/fonc.2026.1903894"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2026.1903894","relation":{},"ISSN":["2234-943X"],"issn-type":[{"value":"2234-943X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,1]]},"article-number":"1903894"}}