{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T15:33:28Z","timestamp":1779291208664,"version":"3.51.4"},"reference-count":46,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2025,12,23]],"date-time":"2025-12-23T00:00:00Z","timestamp":1766448000000},"content-version":"vor","delay-in-days":1,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100008658","name":"DKFZ","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008658","id-type":"DOI","asserted-by":"publisher"}]},{"name":"City of Vienna Fund for Innovative Interdisciplinary Cancer Research"},{"name":"Forschungsgesellschaft f\u00fcr Cerebrale Tumore"},{"DOI":"10.13039\/501100002203","name":"Brain Tumour Charity","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002203","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Children\u2019s National Hospital, Brain Tumor Institute"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,4,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Astroblastomas are rare brain tumors predominantly affecting children and young adults, for which molecular subtypes and clinical management remain undefined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We analyzed tumor samples, molecular profiles, and clinical data from 200 patients, classified as \u201cAstroblastoma, MN1-altered\u201d under WHO criteria, using DNA methylation profiling, DNA\/RNA profiling\/sequencing, and survival analyses.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>DNA methylation analyses identified 3 groups: Group A (n\u2009=\u2009143, characterized by MN1::BEND2 fusions, predominantly supratentorial location, with striking female predominance and favorable survival); Group B (n\u2009=\u200937, epigenetically and transcriptionally closely related to Group A, but characterized by EWSR1::BEND2 fusions, with spinal and infratentorial locations and poor prognosis); and Group C (n\u2009=\u200920, epigenetically and transcriptionally distinct, characterized by MN1::CXXC5 fusions, exclusively supratentorially located, with favorable survival). Progression-free and overall survival were significantly shorter in Group B (5-year PFS 14%; 10-year OS 54%) compared to A (5-year PFS 47%; 10-year OS 89%) and C (5-year PFS 75%; 10-year OS 89%). Radiotherapy improved PFS in Group B (hazard ratio 0.25), while no clear benefit was identified for Groups A and C.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Astroblastoma, MN1-altered, comprises 3 molecularly and clinically distinct groups, characterized by different fusion genes, including those without MN1. These new insights, including the identification of potential predictive biomarkers like 14q\/16q loss, provide a framework for the development of risk-stratified therapeutic approaches. Importantly, we identified a molecularly defined high-risk group that benefits from radiation therapy. Our findings redefine Astroblastoma as a molecularly diverse tumor type, propose a refined classification, support the development of risk-adapted therapeutic strategies and provide a rational standard of care.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/neuonc\/noaf283","type":"journal-article","created":{"date-parts":[[2025,12,18]],"date-time":"2025-12-18T13:07:05Z","timestamp":1766063225000},"page":"1005-1019","source":"Crossref","is-referenced-by-count":2,"title":["Molecular and clinical stratification of astroblastomas: Three distinct fusion-defined groups informing risk-adapted treatment strategies"],"prefix":"10.1093","volume":"28","author":[{"given":"Aniello","family":"Federico","sequence":"first","affiliation":[{"name":"Hopp-Children\u2019s Cancer Center Heidelberg (KiTZ) , Heidelberg,","place":["Germany"]},{"name":"Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK) , Heidelberg,","place":["Germany"]},{"name":"Department of Pediatric Hematology and Oncology, Heidelberg University Hospital and National Center for Tumor Diseases (NCT) , Heidelberg,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Felix","family":"Schmitt-Hoffner","sequence":"additional","affiliation":[{"name":"Hopp-Children\u2019s Cancer Center Heidelberg (KiTZ) , Heidelberg,","place":["Germany"]},{"name":"Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK) , Heidelberg,","place":["Germany"]},{"name":"Department of Pediatric Hematology and Oncology, Heidelberg University Hospital and National Center for Tumor Diseases (NCT) , Heidelberg,","place":["Germany"]},{"name":"Faculty of Biosciences, Heidelberg University , Heidelberg,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6382-6833","authenticated-orcid":false,"given":"Adriana","family":"Fonseca","sequence":"additional","affiliation":[{"name":"Brain Tumor Institute, Children\u2019s National Hospital , Washington, District of Columbia, (Ad.F., Lan.W., R.J.P.)","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Neal","family":"Geisemeyer","sequence":"additional","affiliation":[{"name":"Hopp-Children\u2019s Cancer Center Heidelberg (KiTZ) , Heidelberg,","place":["Germany"]},{"name":"Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK) , Heidelberg,","place":["Germany"]},{"name":"Department of Pediatric Hematology and Oncology, Heidelberg University Hospital and National Center for Tumor Diseases (NCT) , Heidelberg,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katharina","family":"Bruckner","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Adolescent Medicine, Comprehensive Cancer Center and Comprehensive Center for Pediatrics, Medical University of Vienna , Vienna,","place":["Austria"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Monika","family":"Mauermann","sequence":"additional","affiliation":[{"name":"Hopp-Children\u2019s Cancer Center Heidelberg (KiTZ) , Heidelberg,","place":["Germany"]},{"name":"Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK) , Heidelberg,","place":["Germany"]},{"name":"Department of Pediatric Hematology and Oncology, Heidelberg University Hospital and National Center for Tumor Diseases (NCT) , Heidelberg,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7616-7665","authenticated-orcid":false,"given":"Martin","family":"Sill","sequence":"additional","affiliation":[{"name":"Hopp-Children\u2019s Cancer Center Heidelberg (KiTZ) , Heidelberg,","place":["Germany"]},{"name":"Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK) , Heidelberg,","place":["Germany"]},{"name":"Department of Neuropathology, Heidelberg University Hospital , Heidelberg,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Damian","family":"Stichel","sequence":"additional","affiliation":[{"name":"Division of Pediatric Glioma Research, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK) , 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