{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T07:27:23Z","timestamp":1791358043970,"version":"4.3.1"},"reference-count":98,"publisher":"Oxford University Press (OUP)","issue":"Supplement_2","license":[{"start":{"date-parts":[[2023,12,15]],"date-time":"2023-12-15T00:00:00Z","timestamp":1702598400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,5,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>H3 K27M-mutant diffuse glioma is a recently identified brain tumor associated with poor prognosis. As of 2016, it is classified by the World Health Organization as a distinct form of grade IV glioma. Despite recognition as an important prognostic and diagnostic feature in diffuse glioma, radiation remains the sole standard of care and no effective systemic therapies are available for H3K27M mutant tumors. This review will detail treatment interventions applied to diffuse midline glioma and diffuse intrinsic pontine glioma (DIPG) prior to the identification of the H3 K27M mutation, the current standard-of-care for H3 K27M-mutant diffuse glioma treatment, and ongoing clinical trials listed on www.clinicaltrials.gov evaluating novel therapeutics in this population. Current clinical trials were identified using clinicaltrials.gov, and studies qualifying for this analysis were active or ongoing interventional trials that evaluated a therapy in at least 1 treatment arm or cohort comprised exclusively of patients with DIPG and H3 K27M-mutant glioma. Forty-one studies met these criteria, including trials evaluating H3 K27M vaccination, chimeric antigen receptor T-cell therapy, and small molecule inhibitors. Ongoing evaluation of novel therapeutics is necessary to identify safe and effective interventions in this underserved patient population.<\/jats:p>","DOI":"10.1093\/neuonc\/noad220","type":"journal-article","created":{"date-parts":[[2023,11,13]],"date-time":"2023-11-13T17:13:24Z","timestamp":1699895604000},"page":"S110-S124","source":"Crossref","is-referenced-by-count":42,"title":["H3 K27M-altered glioma and diffuse intrinsic pontine glioma: Semi-systematic review of treatment landscape and future directions"],"prefix":"10.1093","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5710-5127","authenticated-orcid":false,"given":"Martin","family":"van den Bent","sequence":"first","affiliation":[{"name":"Brain Tumor Center at Erasmus MC Cancer Institute, University Medical Center Rotterdam , Rotterdam , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda M","family":"Saratsis","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Advocate Children\u2019s Hospital , Park Ridge, Illinois , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marjolein","family":"Geurts","sequence":"additional","affiliation":[{"name":"Brain Tumor Center at Erasmus MC Cancer Institute, University Medical Center Rotterdam , Rotterdam , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9332-4677","authenticated-orcid":false,"given":"Enrico","family":"Franceschi","sequence":"additional","affiliation":[{"name":"Department of Nervous System Medical Oncology, IRCCS Istituto delle Scienze Neurologiche di Bologna , Bologna , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,12,15]]},"reference":[{"issue":"3","key":"2024050310322801300_CIT0001","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1007\/s11060-022-04027-2","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","journal-title":"J Neuro Oncol."},{"issue":"4","key":"2024050310322801300_CIT0002","doi-asserted-by":"crossref","first-page":"799","DOI":"10.1093\/neuonc\/noac198","article-title":"National-level overall survival patterns for molecularly-defined diffuse glioma types in the United States","volume":"25","author":"Ostrom","year":"2023","journal-title":"Neuro Oncol"},{"issue":"8","key":"2024050310322801300_CIT0003","doi-asserted-by":"crossref","first-page":"1231","DOI":"10.1093\/neuonc\/noab106","article-title":"The 2021 WHO classification of tumors of the central nervous system: a summary","volume":"23","author":"Louis","year":"2021","journal-title":"Neuro Oncol"},{"issue":"11","key":"2024050310322801300_CIT0004","doi-asserted-by":"crossref","first-page":"1547","DOI":"10.1093\/neuonc\/noy070","article-title":"Prospective feasibility and safety assessment of surgical biopsy for patients with newly diagnosed diffuse intrinsic pontine glioma","volume":"20","author":"Gupta","year":"2018","journal-title":"Neuro Oncol"},{"issue":"3","key":"2024050310322801300_CIT0005","doi-asserted-by":"crossref","first-page":"520","DOI":"10.1016\/j.adro.2019.03.009","article-title":"Role of radiation therapy in the management of diffuse intrinsic pontine glioma: a systematic review","volume":"4","author":"Gallitto","year":"2019","journal-title":"Adv Radiat Oncol"},{"issue":"3","key":"2024050310322801300_CIT0006","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1016\/j.ijrobp.2008.05.030","article-title":"The role of hypofractionation radiotherapy for diffuse intrinsic brainstem glioma in children: a pilot study","volume":"73","author":"Janssens","year":"2009","journal-title":"Int J Radiat Oncol Biol Phys."},{"issue":"3","key":"2024050310322801300_CIT0007","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1007\/s11060-011-0542-4","article-title":"Hypofractionated radiotherapy in the treatment of diffuse intrinsic pontine glioma in children: a single institution\u2019s experience","volume":"104","author":"Negretti","year":"2011","journal-title":"J Neuro Oncol."},{"issue":"2","key":"2024050310322801300_CIT0008","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1016\/j.prro.2016.11.005","article-title":"Radiation and subsequent reirradiation outcomes in the treatment of diffuse intrinsic pontine glioma and a systematic review of the reirradiation literature","volume":"7","author":"Freese","year":"2017","journal-title":"Pract. 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