背景:脊柱弥漫性胶质瘤(DGS)-由硬膜内髓内胶质母细胞瘤组成,星形细胞瘤,少突胶质细胞瘤是非常罕见的肿瘤,约占原发性脊髓肿瘤的2%。由于相对缺乏临床结果数据,他们的最佳治疗方案尚不清楚。
目的:提供DGS治疗和结果的最新分析。
方法:使用国家癌症数据库(NCDB)的观察性队列研究,多中心前瞻性收集的肿瘤学结局数据库.还进行了系统的文献综述,以将所得数据与以前的系列进行比较。
方法:2004年至2018年组织学确诊的DGS患者。
方法:长期总生存率和手术后30/90天的短期死亡率,三十天重新接纳,并延长住院时间。
方法:使用Kaplan-Meier估计和多变量Cox比例风险回归评估切除程度和辅助治疗对总生存期的影响。使用单变量和多变量逻辑回归分析协变量及其对短期手术结果的预后影响。
结果:在符合纳入标准的747例中,有439个星形细胞瘤,14少突胶质细胞瘤,和208个胶质母细胞瘤.60%(n=442)的患者接受了辐射,45%(n=324)接受化疗。肿瘤组织学显著影响生存率;胶质母细胞瘤的生存率最差(中位生存时间[MS]:12.3个月),其次是星形细胞瘤(MS:70.8个月)和少突胶质细胞瘤(MS:71.6个月)(p<0.001)。在胶质母细胞瘤(风险比[HR]:0.194,p<0.001)和其他WHO4级肿瘤(HR:0.223,p=0.003)患者中,大体全切除术(GTR)独立地获得了生存益处。辅助化疗也改善了胶质母细胞瘤(HR:0.244,p=0.007)和WHO4级肿瘤(HR:0.252,p<0.001)患者的生存率。系统文献综述确定了14项先前的研究,DGS的综合死亡率为1.3%,这低于NCDB计算的4%的真实世界结果。这种差异可以通过先前发表的文献中的选择偏差来解释,在这些文献中,只有具有良好结果的中心才会发表其结果。
结论:关于DGS的治疗模式和结果的数据仍然很少。我们的分析,迄今为止最大的,证明GTR和辅助治疗独立地改善了某些高级DGS亚组的生存率。这些最佳可用数据为此类患者提供了最佳管理。
Diffuse gliomas of the spine (DGS)-consisting of intradural intramedullary glioblastoma, astrocytoma, and oligodendroglioma-are exceedingly rare tumors that account for about 2% of primary spinal cord tumors. Much is unknown about their optimal treatment regimen due to a relative lack of clinical outcome data.
To provide an updated analysis on treatment and outcomes in DGS.
Observational cohort study using The National Cancer Database (NCDB), a multicenter prospectively collected oncology outcomes database. A systematic literature review was also performed to compare the resulting data to previous series.
Patients with histologically confirmed DGS from 2004 to 2018.
Long-term overall survival and short-term 30/90-day postsurgical mortality, 30-day readmission, and prolonged hospital length of stay.
Impact of extent of resection and adjuvant therapy on overall survival was evaluated using Kaplan-Meier estimates and multivariable Cox proportional hazards regression. Univariate and multivariate logistic regression was used to analyze covariables and their prognostic impact on short-term surgical outcomes.
Of the 747 cases that met inclusion criteria, there were 439 astrocytomas, 14 oligodendrogliomas, and 208 glioblastomas. Sixty percent (n=442) of patients received radiation, and 45% (n=324) received chemotherapy. Tumor histology significantly impacted survival; glioblastoma had the poorest survival (median survival time [MS]: 12.3 months), followed by astrocytoma (MS: 70.8 months) and oligodendroglioma (MS: 71.6 months) (p<.001). Gross total resection (GTR) independently conferred a survival benefit in patients with glioblastoma (hazard ratio [HR]: 0.194, p<0.001) and other WHO grade four tumors (HR: 0.223, p=.003). Adjuvant chemotherapy also improved survival in patients with glioblastoma (HR: 0.244, p=.007) and WHO grade four tumors (HR: 0.252, p<.001). Systematic literature review identified 14 prior studies with a combined DGS mortality rate of 1.3%, which is lower than the 4% real-world outcomes calculated from the NCDB. This difference may be explained by selection biases in previously published literature in which only centers with favorable outcomes publish their results.
There remains a paucity of data regarding treatment paradigms and outcomes for DGS. Our analysis, the largest to date, demonstrates that GTR and adjuvant therapy independently improve survival for certain high-grade subgroups of DGS. This best-available data informs optimal management for such patients.