Long-term survival with IDH wildtype glioblastoma: first results from the ETERNITY Brain Tumor Funders' Collaborative Consortium (EORTC 1419)

Hertler, Caroline and Felsberg, Joerg and Gramatzki, Dorothee and Le Rhun, Emilie and Clarke, Jennifer and Soffietti, Riccardo and Wick, Wolfgang and Chinot, Olivier and Ducray, Francois and Roth, Patrick and McDonald, Kerrie and Hau, Peter and Hottinger, Andreas F. and Reijneveld, Jaap and Schnell, Oliver and Marosi, Christine and Glantz, Michael and Darlix, Amelie and Lombardi, Giuseppe and Krex, Dietmar and Glas, Martin and Reardon, David A. and van den Bent, Martin and Lefranc, Florence and Herrlinger, Ulrich and Razis, Evangelia and Carpentier, Antoine F. and Phillips, Samuel and Ruda, Roberta and Wick, Antje and Tabouret, Emeline and Meyronet, David and Maurage, Claude-Alain and Rushing, Elisabeth and Rapkins, Robert and Bumes, Elisabeth and Hegi, Monika and Weyerbrock, Astrid and Aregawi, Dawit and Gonzalez-Gomez, Christian and Pellerino, Alessia and Klein, Martin and Preusser, Matthias and Bendszus, Martin and Golfinopoulos, Vassilis and von Deimling, Andreas and Gorlia, Thierry and Wen, Patrick Y. and Reifenberger, Guido and Weller, Michael (2023) Long-term survival with IDH wildtype glioblastoma: first results from the ETERNITY Brain Tumor Funders' Collaborative Consortium (EORTC 1419). EUROPEAN JOURNAL OF CANCER, 189: 112913. ISSN 0959-8049, 1879-0852

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Abstract

Background: Median survival with glioblastoma remains in the range of 12 months on population levels. Only few patients survive for more than 5 years. Patient and disease features associated with long-term survival remain poorly defined. Methods: European Organization for Research and Treatment of Cancer (EORTC) 1419 (ETERNITY) is a registry study supported by the Brain Tumor Funders Collaborative in the US and the EORTC Brain Tumor Group. Patients with glioblastoma surviving at least 5 years from diagnosis were identified at 24 sites in Europe, US, and Australia. In patients with isocitrate dehydrogenase (IDH) wildtype tumours, prognostic factors were analysed using the Kaplan-Meier method and the Cox proportional hazards model. A population-based reference cohort was obtained from the Cantonal cancer registry Zurich. Results: At the database lock of July 2020, 280 patients with histologically centrally confirmed glioblastoma (189 IDH wildtype, 80 IDH mutant, 11 incompletely characterised) had been registered. In the IDH wildtype population, median age was 56 years (range 24-78 years), 96 patients (50.8%) were female, 139 patients (74.3%) had tumours with O6-methyl guanine DNA methyltransferase (MGMT) promoter methylation. Median overall survival was 9.9 years (95% confidence interval [95% CI] 7.9-11.9). Patients without recurrence ex-perienced longer median survival (not reached) than patients with one or more recurrences (8.92 years) (p < 0.001) and had a high rate (48.8%) of MGMT promoter-unmethylated tumours. Conclusions: Freedom from progression is a powerful predictor of overall survival in long-term survivors with glioblastoma. Patients without relapse often have MGMT promoter-unmethylated glioblastoma and may represent a distinct subtype of glioblastoma. & COPY; 2023 The Author(s). Published by Elsevier Ltd. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

Item Type: Article
Uncontrolled Keywords: CENTRAL-NERVOUS-SYSTEM; TEMOZOLOMIDE; RADIOTHERAPY; CLASSIFICATION; MUTATIONS; IDH; MGMT; Prognosis; Outcome; Registry; Wildtype
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Neurologie
Depositing User: Dr. Gernot Deinzer
Date Deposited: 31 Jan 2024 07:13
Last Modified: 31 Jan 2024 07:13
URI: https://pred.uni-regensburg.de/id/eprint/60980

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