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IDH 野生型胶质母细胞瘤的长期生存:来自 EORTC 1419 肿瘤基金研究协作组的首次结果。

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

机构信息

Department of Neurology, University Hospital and University of Zurich, Zurich, Switzerland; Clinical Neuroscience Center, University Hospital and University of Zurich, Zurich, Switzerland.

Institute of Neuropathology, Medical Faculty, Heinrich Heine University and University Hospital Düsseldorf, Düsseldorf, Germany.

出版信息

Eur J Cancer. 2023 Aug;189:112913. doi: 10.1016/j.ejca.2023.05.002. Epub 2023 May 8.

DOI:10.1016/j.ejca.2023.05.002
PMID:37277265
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 O-methylguanine DNA methyltransferase (MGMT) promoter methylation. Median overall survival was 9.9 years (95% confidence interval [95% CI] 7.9-11.9). Patients without recurrence experienced 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.

摘要

背景

胶质母细胞瘤患者的中位生存期仍在人群水平上维持在 12 个月左右。只有少数患者能存活 5 年以上。与长期生存相关的患者和疾病特征仍未得到明确界定。

方法

欧洲癌症研究与治疗组织(EORTC)1419(ETERNITY)是一项在美国和欧洲癌症研究与治疗组织脑肿瘤组的脑肿瘤基金协作组织支持下开展的注册研究。在欧洲、美国和澳大利亚的 24 个地点,确定了至少从诊断起存活 5 年以上的胶质母细胞瘤患者。在异柠檬酸脱氢酶(IDH)野生型肿瘤患者中,使用 Kaplan-Meier 方法和 Cox 比例风险模型分析预后因素。从苏黎世州癌症登记处获得了基于人群的参考队列。

结果

在 2020 年 7 月数据库锁定时,已登记了 280 例经组织学中心确认的胶质母细胞瘤患者(189 例 IDH 野生型,80 例 IDH 突变型,11 例不完全特征)。在 IDH 野生型人群中,中位年龄为 56 岁(范围 24-78 岁),96 例(50.8%)为女性,139 例(74.3%)肿瘤的 O-甲基鸟嘌呤 DNA 甲基转移酶(MGMT)启动子有甲基化。中位总生存期为 9.9 年(95%置信区间[95%CI]7.9-11.9)。无复发患者的中位生存期(未达到)长于有一次或多次复发的患者(8.92 年)(p<0.001),且有较高比例(48.8%)的 MGMT 启动子未甲基化肿瘤。

结论

胶质母细胞瘤长期生存者无进展是总生存期的有力预测指标。无复发患者常伴有 MGMT 启动子未甲基化的胶质母细胞瘤,可能代表胶质母细胞瘤的一个独特亚型。

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