{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T10:59:21Z","timestamp":1794049161274,"version":"4.8.0"},"reference-count":41,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T00:00:00Z","timestamp":1780444800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Emmy-Noether Programme","award":["ID SU 1548\/1-1"],"award-info":[{"award-number":["ID SU 1548\/1-1"]}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["404521405\u2014SFB 1389"],"award-info":[{"award-number":["404521405\u2014SFB 1389"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"name":"German Cancer Aid through the funding instrument \u201cMildred-Scheel-Doctoral Program\u201d","award":["70117260"],"award-info":[{"award-number":["70117260"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,9,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Replication repair deficiency is associated with increased risk of developing malignant gliomas. The aim of this study was to investigate primary mismatch repair deficient gliomas (PMMRDGs), a group of IDH-wildtype and H3-wildtype gliomas that is enriched among patients with CMMRD and Lynch syndrome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We investigated how PMMRDGs differ from other gliomas with respect to DNA methylation profile, genomic alterations, histopathology, and clinical outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>PMMRDGs occur in pediatric, adolescents and the elderly, falling in two related methylation clusters and are characterized by a high frequency of replication repair deficiency. Histology showed multinucleated giant cells, and immunohistochemistry demonstrated loss of MMR protein expression. Survival analysis revealed long-term survival in patients with high mutational burden (&amp;gt;50 mut\/Mb) and an intact chromosome 9p region, which was validated in an independent reference cohort.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Overall, our findings indicate that PMMRDGs represent a distinct type of IDH-wildtype gliomas with potential for long-term survival likely driven by immune activation.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/neuonc\/noag132","type":"journal-article","created":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T11:36:47Z","timestamp":1780400207000},"page":"2153-2165","source":"Crossref","is-referenced-by-count":2,"title":["Primary mismatch repair deficient glioma, IDH-wildtype and H3-wildtype: A giant cell tumor with potential for long-term survival occurring at all ages"],"prefix":"10.1093","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-8394-1905","authenticated-orcid":false,"given":"Abigail K","family":"Suwala","sequence":"first","affiliation":[{"name":"Department of Neuropathology, Institute of Pathology, University Hospital Heidelberg, Heidelberg University , Heidelberg,","place":["Germany"]},{"name":"Clinical Cooperation Unit Neuropathology, German Consortium for Translational Cancer Research (DKTK), German Cancer Research Center (DKFZ) , Heidelberg,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-5067-3009","authenticated-orcid":false,"given":"Dennis","family":"Friedel","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Institute of Pathology, University Hospital Heidelberg, Heidelberg University , Heidelberg,","place":["Germany"]},{"name":"Clinical Cooperation Unit Neuropathology, German Consortium for Translational Cancer Research (DKTK), German Cancer Research Center (DKFZ) , Heidelberg,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix E","family":"Hinz","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Institute of Pathology, University Hospital Heidelberg, Heidelberg University , Heidelberg,","place":["Germany"]},{"name":"Clinical Cooperation Unit Neuropathology, German Consortium for Translational Cancer Research (DKTK), German Cancer Research Center (DKFZ) , Heidelberg,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-8499-7188","authenticated-orcid":false,"given":"Philipp D","family":"Mahlknecht","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Institute of Pathology, University Hospital Heidelberg, Heidelberg University , Heidelberg,","place":["Germany"]},{"name":"Clinical Cooperation Unit Neuropathology, German Consortium for 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