{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T18:17:18Z","timestamp":1788459438464,"version":"build-2803163510"},"reference-count":26,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2021,6,11]],"date-time":"2021-06-11T00:00:00Z","timestamp":1623369600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,1,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Diffuse intrinsic pontine gliomas (DIPG) generally occur in young school-age children, although can occur in adolescents and young adults. The purpose of this study was to describe clinical, radiological, pathologic, and molecular characteristics in patients \u226510 years of age with DIPG enrolled in the International DIPG Registry (IDIPGR).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients \u226510 years of age at diagnosis enrolled in the IDIPGR with imaging confirmed DIPG diagnosis were included. The primary outcome was overall survival (OS) categorized as long-term survivors (LTS) (\u226524 months) or short-term survivors (STS) (&amp;lt;24 months).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Among 1010 patients, 208 (21%) were \u226510 years of age at diagnosis; 152 were eligible with a median age of 12 years (range 10-26.8). Median OS was 13 (2-82) months. The 1-, 3-, and 5-year OS was 59.2%, 5.3%, and 3.3%, respectively. The 18\/152 (11.8%) LTS were more likely to be older (P &amp;lt; .01) and present with longer symptom duration (P &amp;lt; .01). Biopsy and\/or autopsy were performed in 50 (33%) patients; 77%, 61%, 33%, and 6% of patients tested had H3K27M (H3F3A or HIST1H3B), TP53, ATRX, and ACVR1 mutations\/genome alterations, respectively. Two of 18 patients with IDH1 testing were IDH1-mutant and 1 was a LTS. The presence or absence of H3 alterations did not affect survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Patients \u226510 years old with DIPG have a median survival of 13 months. LTS present with longer symptom duration and are likely to be older at presentation compared to STS. ATRX mutation rates were higher in this population than the general DIPG population.<\/jats:p><\/jats:sec>","DOI":"10.1093\/neuonc\/noab140","type":"journal-article","created":{"date-parts":[[2021,6,9]],"date-time":"2021-06-09T12:02:00Z","timestamp":1623240120000},"page":"141-152","source":"Crossref","is-referenced-by-count":23,"title":["Characteristics of patients \u226510 years of age with diffuse intrinsic pontine glioma: a report from the International DIPG\/DMG Registry"],"prefix":"10.1093","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1394-7167","authenticated-orcid":false,"given":"Craig","family":"Erker","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Dalhousie University and IWK Health Centre, Halifax, Nova Scotia, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5522-8112","authenticated-orcid":false,"given":"Adam","family":"Lane","sequence":"additional","affiliation":[{"name":"Division of Bone Marrow Transplantation and Immune Deficiency, Cancer and Blood Diseases Institute, Cincinnati Children\u2019s Hospital Medical Center, Cincinnati, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brooklyn","family":"Chaney","sequence":"additional","affiliation":[{"name":"Division of Oncology, Cancer and Blood Diseases Institute, Cincinnati Children\u2019s Hospital Medical Center, Cincinnati, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0225-6184","authenticated-orcid":false,"given":"Sarah","family":"Leary","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Seattle Children\u2019s Hospital, University of Washington and Fred Hutchinson Cancer Research Center, Seattle, Washington, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9069-8761","authenticated-orcid":false,"given":"Jane E","family":"Minturn","sequence":"additional","affiliation":[{"name":"Division of Oncology, Children\u2019s Hospital of Philadelphia and Perelman School of Medicine, Philadelphia, Pennsylvania, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2112-5251","authenticated-orcid":false,"given":"Ute","family":"Bartels","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Oncology, University of Toronto and The Hospital for Sick Children, Toronto, Ontario, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9413-7531","authenticated-orcid":false,"given":"Roger J","family":"Packer","sequence":"additional","affiliation":[{"name":"Department of Neurology, Center for 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