j - JOUR T1 -原发性脊髓胶质母细胞瘤的自然病程和预后JF - Neurology - JO - Neurology - SP - e1497 LP 首页- e1509 DO - 10.1212/WNL.0000000000206834六世- 100 - 14 AU - Aymeric麦洛盟Louis-Marie梗AU -伯特兰Mathon AU -克利斯朵夫Joubert盟Thiebaud Picart AU -文森特Jecko AU - Luc Bauchet盟Florian伯纳德盟Xavier卡斯特尔AU -路易Chenin盟Ann-Rose库克AU -伊芙金刚砂AU -多米尼克•Figarella-Branger盟Guillaume Gauchotte AU -托马斯Graillon AU -安妮Jouvet AU -米歇尔Kalamarides AU -史蒂文Knafo盟Arnaud Lazard AU -文森特Lubrano盟(Karima Mokhtari称非盟-瓦莱丽Rigau盟文森特RoualdesAU - Audrey Rousseau - Romuald Seizeur - Emmanuelle eur - coste AU - Jimmy Voirin AU - Philippe Metellus AU - Johan Pallud AU - Ilyess Zemmoura AU -以及髓质胶质母细胞瘤研究小组Y1 - 2023/04/04 UR - //www.ez-admanager.com/content/100/14/e1497首页.abstract背景和目的原发性脊髓胶质母细胞瘤(PsGBM)极为罕见。PsGBM严重的神经功能恶化和不可切除性使其成为一种特别致残的恶性肿瘤。由于它是一种罕见且异质性的疾病，对预后因素的评估仍然有限。方法回顾性分析来自法国脑肿瘤数据库和法国神经外科学会神经肿瘤俱乐部的PsGBMs。纳入标准为诊断时年龄≥18岁，脊柱位置，根据2016年世界卫生组织分类新发胶质母细胞瘤的组织病理学诊断，以及2004年至2016年的手术处理。通过集中神经病理检查确诊。主要终点是总生存期(OS)。还收集了治疗干预措施和神经学结果。Results Thirty-three patients with a histopathologically confirmed PsGBM (median age 50.9 years) were included (27 centers). The median OS was 13.1 months (range 2.5–23.7), and the median progression-free survival was 5.9 months (range 1.6–10.2). In multivariable analyses using Cox model, Eastern Cooperative Oncology Group (ECOG) performance status at 0–1 was the only independent predictor of longer OS (hazard ratio [HR] 0.13, 95% CI 0.02–0.801; p = 0.02), whereas a Karnofsky performance status (KPS) score <60 (HR 2.89, 95% CI 1.05–7.92; p = 0.03) and a cervical anatomical location (HR 4.14, 95% CI 1.32–12.98; p = 0.01) were independent predictors of shorter OS. The ambulatory status (Frankel D–E) (HR 0.38, 95% CI 0.07–1.985; p = 0.250) was not an independent prognostic factor, while the concomitant standard radiochemotherapy with temozolomide (Stupp protocol) (HR 0.35, 95% CI 0.118–1.05; p = 0.06) was at the limit of significance.Discussion Preoperative ECOG performance status, KPS score, and the location are independent predictors of OS of PsGBMs in adults. Further analyses are required to capture the survival benefit of concomitant standard radiochemotherapy with temozolomide.CT=chemotherapy; ECOG=Eastern Cooperative Oncology Group; FBTDB=French Brain Tumor Database; FLAIR=fluid-attenuated inversion recovery; FU=follow-up; GBM=glioblastoma; HR=hazard ratio; IDH=isocitrate dehydrogenase; KPS=Karnofsky performance status; MGMT=O(6)-methylguanine-DNA methyltransferase; OS=overall survival; PFS=progression-free survival; PsGBM=primary spinal glioblastoma; RENOCLIP=Réseau de Neuro-Oncologie CLInico Pathologique; RENOP=Réseau de Neuro-oncologie pathologique; RT=radiotherapy; STR=subtotal resection surgery; TERT=telomerase reverse transcriptase; WHO=World Health Organization ER -