RT期刊文章SR电子T1与MAPK抑制肿瘤持续控制BRAFV600-Mutant成人神经胶质和Glioneuronal肿瘤摩根富林明神经病学神经学乔FD Lip首页pincott Williams &威尔金斯SP e673 OP e683 10.1212 / WNL。97签证官0000000000012330是7 A1会Berzero A1路易莎Bellu A1卡普辛巴尔迪尼A1弗朗索瓦·盖恩A1 Ducray A1大卫Marica Eoli A1安东尼奥Silvani A1卡罗琳Dehais A1 Ahmed Idbaih A1 Nadia Younan A1卢多维奇Nguyen-Them A1 Stephan盖拉德A1 Francesco Pasqualetti A1 Coralie Lepage-Seydoux A1萨金娜Sekkate A1帕特里夏·特雷斯卡A1 Aurelie ka A1朱莉Gratieux A1萨米Ammari A1爱德华Saragoussi A1朱利安Savatovsky A1让Delattre A1 Khe Hoang-Xuan A1大卫Meyronet A1奇亚拉别墅A1弗兰克Bielle A1 Marc桑丘A1 Mehdi Touat A1安娜路易莎迪斯蒂法诺年2021 UL //www.ez-admanager.com/content/97/7/e673.abstract AB客观评估英国皇家空军和MEK抑制剂(拉菲/ MEKi)是否可以提供长期的成人患者的临床效益BRAF V600-mutant胶质和glioneuronal肿瘤(GGNTs),分析肿瘤反应和长期结果回顾性队列。首页方法我们进行了一个回顾神经肿瘤学学会举办6部门的机构数据库搜索成人患者复发性或传播BRAF V600-mutant GGNTs RAFi / MEKi对待。结果28成人患者复发或传播BRAF V600-mutant报告(n = 9),多形性xanthoastrocytomas (n = 9),和弥漫性神经胶质瘤(n = 10)包括在这项研究。当时与拉菲/ MEKi治疗开始,所有肿瘤显示高档肿瘤放射特性。13名患者接受作为单药拉菲(vemurafenib (n = 11) dabrafenib [n = 2]),和15收到拉菲的组合/ MEKi (vemurafenib + cobimetinib (n = 5) dabrafenib + trametinib [n = 10])。11个病人取得了部分或完全缓解(11 28,39%),平均减少−78%的肿瘤负荷。急救员经验值增加10分的Karnofsky性能状态(KPS)评分和18个月的中位无进展生存,这是超过一线治疗(即实现。7个月,p = 0.047)。急救员最好KPS评分(p = 0.018)和倾向于更年轻(p = 0.061)和治疗早期nonresponders相比(p = 0.099)。五个病人重新拉菲/ MEKi进展,与小说肿瘤反应2。在单变量和多变量分析,对拉菲/ MEKi总体生存的独立预测指标。结论我们的研究凸显了长期的临床益处RAFi /成人患者的MEKi BRAF V600-mutant GGNTs并鼓励重新反应者。Classification of Evidence This study provides Class III evidence that, for adult patients with BRAF V600-mutant GGNT, RAFi/MEKi can reduce tumor burden and provide clinical benefit.CR=complete response; GGNT=glial and glioneuronal tumor; KPS=Karnofsky Performance Status; MEKi=MEK inhibitors; MGMT=O6-methylguanine-DNA methyltransferase; OS=overall survival; PD=progressive disease; PFS=progression-free survival; PR=partial response; PXA=pleomorphic xanthoastrocytoma; RAFi=RAF inhibitors; RANO=Response Assessment in Neuro-Oncology; SD=stable disease