随着神经胶质瘤的分类、风险分层和护理标准的重大转变，我们试图了解分子特征、临床指标和接受的治疗如何影响这些肿瘤患者的总体生存率。我们收集了一组患者来自癌症基因组图谱、基因组学证据肿瘤信息交换项目和丹纳法伯癌症研究所/布莱根妇女医院的组织病理学诊断的神经胶质瘤。该研究结合了回顾性临床、组织学和分子数据以及对患者生存的前瞻性评估。共鉴定出 4,400 例神经胶质瘤：2,195 例胶质母细胞瘤、1,198 例 IDH1/2 突变型星形细胞瘤、531 例少突神经胶质瘤、271 例其他 IDH1/2 野生型神经胶质瘤和 205 例儿童型神经胶质瘤神经胶质瘤。分子分类更新了 27.2% 的神经胶质瘤的原始组织病理学诊断。检查神经胶质瘤亚型之间分子改变的分布揭示了致瘤途径中相互排斥的改变。与 TCGA 患者相比，非 TCGA 患者的总生存期显着改善，胶质母细胞瘤、IDH1/2 突变星形细胞瘤和少突胶质细胞瘤的生存期分别延长 26.7%、55.6% 和 127.8%（均 p<0.01）。研究人员对几个预后特征进行了表征，包括胶质母细胞瘤中的 NF1 改变和 21q 缺失，以及 IDH1/2 突变型星形细胞瘤中的 EGFR 扩增和 22q 缺失。利用该队列的规模，生成列线图来评估基于患者年龄、肿瘤的分子特征和接受的治疗的总体生存概率。通过应用现代分子标准，我们描述了神经胶质瘤亚型的基因组多样性，识别临床适用的预后特征，并提供患者生存的最新更新，作为正在进行的研究的参考。© 作者 2024。由牛津大学出版社代表神经肿瘤学会出版。

With the significant shift in the classification, risk stratification, and standards of care for gliomas, we sought to understand how the overall survival of patients with these tumors is impacted by molecular features, clinical metrics, and treatment received.We assembled a cohort of patients with a histopathologically diagnosed glioma from The Cancer Genome Atlas, Project Genomics Evidence Neoplasia Information Exchange, and Dana-Farber Cancer Institute/Brigham and Women's Hospital. This incorporated retrospective clinical, histological, and molecular data alongside prospective assessment of patient survival.4,400 gliomas were identified: 2,195 glioblastoma, 1,198 IDH1/2-mutant astrocytoma, 531 oligodendroglioma, 271 other IDH1/2-wildtype glioma, and 205 pediatric-type glioma. Molecular classification updated 27.2% of gliomas from their original histopathologic diagnosis. Examining the distribution of molecular alterations across glioma subtypes revealed mutually exclusive alterations within tumorigenic pathways. Non-TCGA patients had significantly improved overall survival compared to TCGA patients, with 26.7%, 55.6%, and 127.8% longer survival for glioblastoma, IDH1/2-mutant astrocytoma, and oligodendroglioma respectively (all p<0.01). Several prognostic features were characterized, including NF1 alteration and 21q loss in glioblastoma, and EGFR amplification and 22q loss in IDH1/2-mutant astrocytoma. Leveraging the size of this cohort, nomograms were generated to assess the probability of overall survival based on patient age, the molecular features of a tumor, and the treatment received.By applying modern molecular criteria, we characterize the genomic diversity across glioma subtypes, identify clinically applicable prognostic features, and provide a contemporary update on patient survival to serve as a reference for ongoing investigations.© The Author(s) 2024. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.