BRAF基因突变是影响儿童低级别胶质瘤的重要致病因子，然而BRAF基因改变对成年胶质瘤的临床进程和治疗反应的含义仍不清楚。在本文中，我们采用临床、病理/分子和转归数据，对超过300例BRAF-mutated胶质瘤患者进行多机构队列特征鉴定，其中包括超过200名成年患者。我们观察到成年人和儿童BRAF-mutant胶质瘤具有明显不同的临床和分子特征，儿童肿瘤中BRAFV600E（Class I）和BRAF融合的发生率更高。BRAFV600E突变在成年胶质瘤整体中与生存率的提高相关，但在胶质母细胞瘤中不相关。观察到的其他基因组突变在功能类别内是与BRAF突变类别在胶质瘤发病学中的潜在角色相一致。在我们的成年队列中，BRAFV600E突变表现出对靶向治疗的敏感性。总的来说，这个庞大的BRAF改变的成年胶质瘤队列呈现出广泛的分子变化，并对治疗敏感性和生存率产生了影响。©2023. 作者（们）。

BRAF mutations are a significant driver of disease in pediatric low-grade glioma, but the implications of BRAF alterations on the clinical course and treatment response in adult glioma remain unclear. Here, we characterize a multi-institutional cohort of more than 300 patients (>200 adults) with BRAF-mutated glioma using clinical, pathological/molecular, and outcome data. We observed that adult and pediatric BRAF-mutant gliomas harbor distinct clinical and molecular features, with a higher prevalence of BRAFV600E (Class I) and BRAF fusions in pediatric tumors. BRAFV600E alterations were associated with improved survival in adults with glioma overall, though not in glioblastoma. Other genomic alterations observed within functional classes were consistent with the putative roles of those BRAF mutation classes in glioma pathogenesis. In our adult cohort, BRAFV600E alterations conferred sensitivity to targeted therapies. Overall, this large cohort of BRAF-altered adult gliomas demonstrates a broad range of molecular alterations with implications for treatment sensitivity and survival.© 2023. The Author(s).