{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,29]],"date-time":"2026-01-29T22:37:19Z","timestamp":1769726239549,"version":"3.49.0"},"reference-count":68,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"24","license":[{"start":{"date-parts":[[2025,12,15]],"date-time":"2025-12-15T00:00:00Z","timestamp":1765756800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Neurosurg Case Lessons"],"published-print":{"date-parts":[[2025,12,15]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND<\/jats:title>\n                    <jats:p>H3 K27\u2013altered diffuse midline gliomas (DMGs) are a rare form of primary CNS tumors. In this retrospective single-center case study, DMGs were reviewed for clinical and imaging findings, surgical approaches and challenges, and molecular diagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>OBSERVATIONS<\/jats:title>\n                    <jats:p>Four cases of adult DMG, H3 K27\u2013altered, located among midline structures of the thalamus, brainstem, and spinal cord are presented here. All tumors exhibited heterogeneous presentations on imaging. Symptoms ranged from unspecific back pain and vertigo to focal neurological deficits. Surgery was complicated by high vascularization, infiltrative growth, and proximity to eloquent areas. Diagnostic accuracy was increased by epigenetic DNA methylation\u2013based classification. Three cases were rapidly progressive and resulted in death within 1 year of diagnosis. One case had an exceptionally long overall survival of &gt; 5 years, which was associated with a FGFR1 p.N546K hotspot mutation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>LESSONS<\/jats:title>\n                    <jats:p>DMGs are rare but imitate other pathologies due to variable clinical and radiological characteristics. Surgery is complicated by location and high vascularization. Although DMGs are rare, they should be considered as a differential diagnosis in intracranial and spinal masses in adults. As the FGFR1 p.N546K hotspot mutation is associated with prolonged survival, it may justify more radical surgery in eloquent regions.<\/jats:p>\n                    <jats:p>\n                      <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"https:\/\/thejns.org\/doi\/10.3171\/CASE25357\">https:\/\/thejns.org\/doi\/10.3171\/CASE25357<\/jats:ext-link>\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/case25357","type":"journal-article","created":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T11:59:47Z","timestamp":1769083187000},"source":"Crossref","is-referenced-by-count":0,"title":["The great imitator: clinical, surgical, and molecular insights into diffuse midline gliomas, H3 K27\u2013altered. Illustrative cases"],"prefix":"10.3171","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-6729-9341","authenticated-orcid":true,"given":"Jan","family":"Hellmeyer","sequence":"first","affiliation":[{"name":"School of Medicine, Charit\u00e9\u2013Universit\u00e4tsmedizin Berlin"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lennart W.","family":"Sannwald","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Ulm University, Ulm"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mats L.","family":"Moskopp","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Medical Faculty Carl Gustav Carus, Technische Universit\u00e4t Dresden"},{"name":"Institute of Physiology, Medical Faculty Carl Gustav Carus, Technische Universit\u00e4t Dresden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1945-497X","authenticated-orcid":true,"given":"David","family":"Capper","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Charit\u00e9\u2013Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t Berlin and Humboldt-Universit\u00e4t zu Berlin"},{"name":"German Cancer Consortium (DKTK), partner site Berlin, German Cancer Research Centre (DKFZ), Heidelberg"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dag","family":"Moskopp","sequence":"additional","affiliation":[{"name":"Oncological Rehabilitation, Immanuel Hospital M\u00e4rkische Schweiz, Brandenburg Medical School Theodor Fontane, Campus Buckow, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"bib1","series-title":"Diffuse midline glioma, H3 K27\u2013altered. In: WHO Classification of Tumours: Central Nervous System Tumours","first-page":"69","author":"Varlet","year":"2021","unstructured":"Varlet PBaker SJEllison DWDiffuse midline glioma, H3 K27\u2013altered. In: WHO Classification of Tumours: Central Nervous System Tumours. 5th ed. International Agency for Research on Cancer; 2021:69-73."},{"issue":"1","key":"bib2","series-title":"Nat Commun","first-page":"2146","article-title":"PFA ependymoma-associated protein EZHIP inhibits PRC2 activity through a H3 K27M-like mechanism","volume":"10","author":"Jain","year":"2019","unstructured":"Jain SUDo TJLund PJPFA ependymoma-associated protein EZHIP inhibits PRC2 activity through a H3 K27M-like mechanism. 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Acta Neuropathol. 2020;139(6):1071-1088."},{"issue":"21","key":"bib4","series-title":"Cancers (Basel)","first-page":"5280","article-title":"New developments in the pathogenesis, therapeutic targeting, and treatment of H3K27M-mutant diffuse midline glioma","volume":"13","author":"Argersinger","year":"2021","unstructured":"Argersinger DPRivas SRShah AHJackson SHeiss JD.New developments in the pathogenesis, therapeutic targeting, and treatment of H3K27M-mutant diffuse midline glioma. Cancers (Basel). 2021;13(21):5280."},{"issue":"6","key":"bib5","series-title":"Acta Neuropathol","first-page":"803","article-title":"The 2016 World Health Organization classification of tumors of the central nervous system: a summary","volume":"131","author":"Louis","year":"2016","unstructured":"Louis DNPerry AReifenberger GThe 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol. 2016;131(6):803-820."},{"issue":"8","key":"bib6","series-title":"Neuro Oncol","first-page":"1231","article-title":"The 2021 WHO classification of tumors of the central nervous system: a summary","volume":"23","author":"Louis","year":"2021","unstructured":"Louis DNPerry AWesseling PThe 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol. 2021;23(8):1231-1251."},{"issue":"5","key":"bib7","series-title":"Brain Pathol","first-page":"569","article-title":"Diffuse midline gliomas with histone H3-K27M mutation: a series of 47 cases assessing the spectrum of morphologic variation and associated genetic alterations","volume":"26","author":"Solomon","year":"2016","unstructured":"Solomon DAWood MDTihan TDiffuse midline gliomas with histone H3-K27M mutation: a series of 47 cases assessing the spectrum of morphologic variation and associated genetic alterations. Brain Pathol. 2016;26(5):569-580."},{"issue":"suppl 4","key":"bib8","series-title":"Neuro Oncol","first-page":"iv1","article-title":"CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2011-2015","volume":"20","author":"Ostrom","year":"2018","unstructured":"Ostrom QTGittleman HTruitt GBoscia AKruchko CBarnholtz-Sloan JS.CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2011-2015. Neuro Oncol. 2018;20(suppl 4):iv1-iv86."},{"issue":"1","key":"bib9","series-title":"Cancer Treat Rev","first-page":"27","article-title":"Diffuse intrinsic pontine gliomas: a systematic update on clinical trials and biology","volume":"38","author":"Jansen","year":"2012","unstructured":"Jansen MHvan Vuurden DGVandertop WPKaspers GJ.Diffuse intrinsic pontine gliomas: a systematic update on clinical trials and biology. Cancer Treat Rev. 2012;38(1):27-35."},{"issue":"1","key":"bib10","series-title":"Acta Neuropathol Commun","first-page":"93","article-title":"Targeted detection of genetic alterations reveal the prognostic impact of H3K27M and MAPK pathway aberrations in paediatric thalamic glioma","volume":"4","author":"Ryall","year":"2016","unstructured":"Ryall SKrishnatry RArnoldo ATargeted detection of genetic alterations reveal the prognostic impact of H3K27M and MAPK pathway aberrations in paediatric thalamic glioma. Acta Neuropathol Commun. 2016;4(1):93."},{"issue":"6","key":"bib11","series-title":"Am J Surg Pathol","first-page":"863","article-title":"Diffuse midline gliomas with histone H3 K27M mutation in adults and children: A retrospective series of 164 cases","volume":"46","author":"Zheng","year":"2022","unstructured":"Zheng LGong JYu TDiffuse midline gliomas with histone H3 K27M mutation in adults and children: A retrospective series of 164 cases. Am J Surg Pathol. 2022;46(6):863-871."},{"issue":"6","key":"bib12","series-title":"J Neurosurg","first-page":"1662","article-title":"Imaging characteristics of adult H3 K27M-mutant gliomas","volume":"133","author":"Qiu","year":"2019","unstructured":"Qiu TChanchotisatien AQin ZImaging characteristics of adult H3 K27M-mutant gliomas. J Neurosurg. 2019;133(6):1662-1670."},{"issue":"3","key":"bib13","series-title":"J Neurooncol","first-page":"225","article-title":"H3K27M-mutant diffuse midline gliomas should be further molecularly stratified: an integrated analysis of 669 patients","volume":"155","author":"Vuong","year":"2021","unstructured":"Vuong HGLe HTNgo TNMH3K27M-mutant diffuse midline gliomas should be further molecularly stratified: an integrated analysis of 669 patients. J Neurooncol. 2021;155(3):225-234."},{"issue":"3","key":"bib14","series-title":"J Neurooncol","first-page":"405","article-title":"The prognostic significance of HIST1H3B\/C and H3F3A K27M mutations in diffuse midline gliomas is influenced by patient age","volume":"158","author":"Vuong","year":"2022","unstructured":"Vuong HGNgo TNMLe HTDunn IF.The prognostic significance of HIST1H3B\/C and H3F3A K27M mutations in diffuse midline gliomas is influenced by patient age. J Neurooncol. 2022;158(3):405-412."},{"issue":"5","key":"bib15","series-title":"J Neurosurg","first-page":"1307","article-title":"A comprehensive multicenter analysis of clinical, molecular, and imaging characteristics and outcomes of H3 K27-altered diffuse midline glioma in adults","volume":"142","author":"Sim","year":"2025","unstructured":"Sim YMcClelland ACChoi KA comprehensive multicenter analysis of clinical, molecular, and imaging characteristics and outcomes of H3 K27-altered diffuse midline glioma in adults. J Neurosurg. 2025;142(5):1307-1318."},{"issue":"16","key":"bib16","series-title":"J Neurosurg Case Lessons","first-page":"CASE22529","article-title":"Diffuse midline glioma, H3K27-altered, of the conus medullaris presenting as acute urinary retention: illustrative case","volume":"5","author":"Saluja","year":"2023","unstructured":"Saluja SRazzaq BServider JASeidman RMushlin H.Diffuse midline glioma, H3K27-altered, of the conus medullaris presenting as acute urinary retention: illustrative case. J Neurosurg Case Lessons. 2023;5(16):CASE22529."},{"issue":"10","key":"bib17","series-title":"Acta Radiol","first-page":"1404","article-title":"Radiologic findings and the molecular expression profile of diffuse midline glioma H3 K27M mutant","volume":"62","author":"Seong","year":"2021","unstructured":"Seong MKim STNoh JHKim YKKim HJ.Radiologic findings and the molecular expression profile of diffuse midline glioma H3 K27M mutant. Acta Radiol. 2021;62(10):1404-1411."},{"issue":"2","key":"bib18","series-title":"J Neurooncol","first-page":"437","article-title":"Leptomeningeal dissemination in H3 K27M- mutant diffuse midline gliomas: clinical characteristics, risk factors, and prognostic insights","volume":"172","author":"Zhong","year":"2025","unstructured":"Zhong SZuo JFu XLeptomeningeal dissemination in H3 K27M- mutant diffuse midline gliomas: clinical characteristics, risk factors, and prognostic insights. J Neurooncol. 2025;172(2):437-445."},{"key":"bib19","series-title":"Front Neurol","first-page":"1097157","article-title":"Adult spinal cord diffuse midline glioma, H3 K27-altered mimics symptoms of central nervous system infection: a case report","volume":"14","author":"Chen","year":"2023","unstructured":"Chen XLi YBu HZou YHe JLiu H.Adult spinal cord diffuse midline glioma, H3 K27-altered mimics symptoms of central nervous system infection: a case report. Front Neurol. 2023;14:1097157."},{"issue":"6","key":"bib20","series-title":"Neuro Oncol","first-page":"680","article-title":"Thalamic high-grade gliomas in children: a distinct clinical subset?","volume":"13","author":"Kramm","year":"2011","unstructured":"Kramm CMButenhoff SRausche UThalamic high-grade gliomas in children: a distinct clinical subset? Neuro Oncol. June 2011;13(6):680-689."},{"issue":"8","key":"bib21","series-title":"Childs Nerv Syst","first-page":"2229","article-title":"H3 K27-altered diffuse midline glioma presenting as massive cerebellopontine hemorrhage","volume":"39","author":"Azad","year":"2023","unstructured":"Azad TDKalluri ALHansen LJAmmar ACohen AR.H3 K27-altered diffuse midline glioma presenting as massive cerebellopontine hemorrhage. Childs Nerv Syst. 2023;39(8):2229-2232."},{"issue":"1","key":"bib22","series-title":"J Neurooncol","first-page":"87","article-title":"Incidence and clinicopathologic features of H3 K27M mutations in adults with radiographically-determined midline gliomas","volume":"143","author":"Schreck","year":"2019","unstructured":"Schreck KCRanjan SSkorupan NIncidence and clinicopathologic features of H3 K27M mutations in adults with radiographically-determined midline gliomas. J Neurooncol. 2019;143(1):87-93."},{"key":"bib23","series-title":"Clin Neurol Neurosurg","first-page":"108449","article-title":"Thalamic H3K27M altered diffuse midline gliomas: clinicopathological and outcome analysis","volume":"244","author":"Chaturvedi","year":"2024","unstructured":"Chaturvedi ASadashiva NShukla DThalamic H3K27M altered diffuse midline gliomas: clinicopathological and outcome analysis. Clin Neurol Neurosurg. 2024;244:108449."},{"issue":"A","key":"bib24","series-title":"J Clin Neurosci","first-page":"1","article-title":"Adult diffuse midline gliomas: clinical, radiological, and genetic characteristics","volume":"82","author":"Dono","year":"2020","unstructured":"Dono ATakayasu TBallester LYEsquenazi Y.Adult diffuse midline gliomas: clinical, radiological, and genetic characteristics. J Clin Neurosci. 2020;82(A):1-8."},{"issue":"1","key":"bib25","series-title":"Acta Neuropathol","first-page":"2","article-title":"A new subtype of diffuse midline glioma, H3 K27 and BRAF\/FGFR1 co-altered: a clinico-radiological and histomolecular characterisation","volume":"147","author":"Auffret","year":"2023","unstructured":"Auffret LAjlil YTauzi\u00e8de-Espariat AA new subtype of diffuse midline glioma, H3 K27 and BRAF\/FGFR1 co-altered: a clinico-radiological and histomolecular characterisation. 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International Agency for Research on Cancer; 2021:69-73."},{"issue":"1","key":"bib2","series-title":"Nat Commun","first-page":"2146","article-title":"PFA ependymoma-associated protein EZHIP inhibits PRC2 activity through a H3 K27M-like mechanism","volume":"10","author":"Jain","year":"2019","unstructured":"Jain SUDo TJLund PJPFA ependymoma-associated protein EZHIP inhibits PRC2 activity through a H3 K27M-like mechanism. Nat Commun. 2019;10(1):2146."},{"issue":"6","key":"bib3","series-title":"Acta Neuropathol","first-page":"1071","article-title":"Pediatric bithalamic gliomas have a distinct epigenetic signature and frequent EGFR exon 20 insertions resulting in potential sensitivity to targeted kinase inhibition","volume":"139","author":"Mondal","year":"2020","unstructured":"Mondal GLee JCRavindranathan APediatric bithalamic gliomas have a distinct epigenetic signature and frequent EGFR exon 20 insertions resulting in potential sensitivity to targeted kinase inhibition. Acta Neuropathol. 2020;139(6):1071-1088."},{"issue":"21","key":"bib4","series-title":"Cancers (Basel)","first-page":"5280","article-title":"New developments in the pathogenesis, therapeutic targeting, and treatment of H3K27M-mutant diffuse midline glioma","volume":"13","author":"Argersinger","year":"2021","unstructured":"Argersinger DPRivas SRShah AHJackson SHeiss JD.New developments in the pathogenesis, therapeutic targeting, and treatment of H3K27M-mutant diffuse midline glioma. Cancers (Basel). 2021;13(21):5280."},{"issue":"6","key":"bib5","series-title":"Acta Neuropathol","first-page":"803","article-title":"The 2016 World Health Organization classification of tumors of the central nervous system: a summary","volume":"131","author":"Louis","year":"2016","unstructured":"Louis DNPerry AReifenberger GThe 2016 World Health Organization classification of tumors of the central nervous system: a summary. 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Brain Pathol. 2016;26(5):569-580."},{"issue":"suppl 4","key":"bib8","series-title":"Neuro Oncol","first-page":"iv1","article-title":"CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2011-2015","volume":"20","author":"Ostrom","year":"2018","unstructured":"Ostrom QTGittleman HTruitt GBoscia AKruchko CBarnholtz-Sloan JS.CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2011-2015. Neuro Oncol. 2018;20(suppl 4):iv1-iv86."},{"issue":"1","key":"bib9","series-title":"Cancer Treat Rev","first-page":"27","article-title":"Diffuse intrinsic pontine gliomas: a systematic update on clinical trials and biology","volume":"38","author":"Jansen","year":"2012","unstructured":"Jansen MHvan Vuurden DGVandertop WPKaspers GJ.Diffuse intrinsic pontine gliomas: a systematic update on clinical trials and biology. Cancer Treat Rev. 2012;38(1):27-35."},{"issue":"1","key":"bib10","series-title":"Acta Neuropathol Commun","first-page":"93","article-title":"Targeted detection of genetic alterations reveal the prognostic impact of H3K27M and MAPK pathway aberrations in paediatric thalamic glioma","volume":"4","author":"Ryall","year":"2016","unstructured":"Ryall SKrishnatry RArnoldo ATargeted detection of genetic alterations reveal the prognostic impact of H3K27M and MAPK pathway aberrations in paediatric thalamic glioma. Acta Neuropathol Commun. 2016;4(1):93."},{"issue":"6","key":"bib11","series-title":"Am J Surg Pathol","first-page":"863","article-title":"Diffuse midline gliomas with histone H3 K27M mutation in adults and children: A retrospective series of 164 cases","volume":"46","author":"Zheng","year":"2022","unstructured":"Zheng LGong JYu TDiffuse midline gliomas with histone H3 K27M mutation in adults and children: A retrospective series of 164 cases. Am J Surg Pathol. 2022;46(6):863-871."},{"issue":"6","key":"bib12","series-title":"J Neurosurg","first-page":"1662","article-title":"Imaging characteristics of adult H3 K27M-mutant gliomas","volume":"133","author":"Qiu","year":"2019","unstructured":"Qiu TChanchotisatien AQin ZImaging characteristics of adult H3 K27M-mutant gliomas. J Neurosurg. 2019;133(6):1662-1670."},{"issue":"3","key":"bib13","series-title":"J Neurooncol","first-page":"225","article-title":"H3K27M-mutant diffuse midline gliomas should be further molecularly stratified: an integrated analysis of 669 patients","volume":"155","author":"Vuong","year":"2021","unstructured":"Vuong HGLe HTNgo TNMH3K27M-mutant diffuse midline gliomas should be further molecularly stratified: an integrated analysis of 669 patients. J Neurooncol. 2021;155(3):225-234."},{"issue":"3","key":"bib14","series-title":"J Neurooncol","first-page":"405","article-title":"The prognostic significance of HIST1H3B\/C and H3F3A K27M mutations in diffuse midline gliomas is influenced by patient age","volume":"158","author":"Vuong","year":"2022","unstructured":"Vuong HGNgo TNMLe HTDunn IF.The prognostic significance of HIST1H3B\/C and H3F3A K27M mutations in diffuse midline gliomas is influenced by patient age. J Neurooncol. 2022;158(3):405-412."},{"issue":"5","key":"bib15","series-title":"J Neurosurg","first-page":"1307","article-title":"A comprehensive multicenter analysis of clinical, molecular, and imaging characteristics and outcomes of H3 K27-altered diffuse midline glioma in adults","volume":"142","author":"Sim","year":"2025","unstructured":"Sim YMcClelland ACChoi KA comprehensive multicenter analysis of clinical, molecular, and imaging characteristics and outcomes of H3 K27-altered diffuse midline glioma in adults. 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