Department of Radiation Oncology, Columbia University Medical Center, 622 West 168th Street, BNH B011, New York, NY, 10032, USA.
Department of Neurological Surgery, Columbia University Medical Center, 710 West 168th Street, New York, NY, 10032, USA.
J Neurooncol. 2018 May;138(1):173-182. doi: 10.1007/s11060-018-2787-7. Epub 2018 Feb 9.
The World Health Organization (WHO) classification of tumors of the central nervous system (CNS) was recently updated, restructuring solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) into one combined entity. This is the first population-based study to examine outcomes of SFT/HPC based on the new WHO guidelines. The Surveillance, Epidemiology, and End Results (SEER) database (1998-2013) was queried to examine age-adjusted incidence and prognostic factors associated with overall survival in 416 surgically resected cases. Age-adjusted incidence was calculated to be 3.77 per 10,000,000 and was rising. Median survival was 155 months, with 5- and 10-year survival rates of 78 and 61%, respectively. Younger age, Asian/Pacific Islander versus white race, benign histology, tumor location, gross-total resection (GTR), and GTR plus radiation (RT) versus subtotal resection were significantly associated with survival. In multivariable analysis, older age (HR = 1.038, p < 0.0001), infratentorial location (HR = 2.019, p = 0.038), GTR (HR = 0.313, p = 0.041), and GTR + RT (HR = 0.215, p = 0.008) were independent prognostic factors. In the HPC and borderline/malignant subgroups, GTR + RT was associated with significantly increased survival compared with GTR alone (HR = 0.537, p = 0.039 and HR = 0.525, p = 0.038). After eliminating patients that died within 3 months of diagnosis, GTR + RT was still associated with an incremental increase in survival (HR = 0.238, p = 0.031) over GTR alone (HR = 0.280, p = 0.054). GTR + RT may be optimal in the management CNS HPC and SFT/HPC tumors with borderline/malignant features. This study, in combination with existing literature, warrants further investigation of adjuvant radiation through a prospective clinical trial.
世界卫生组织(WHO)中枢神经系统(CNS)肿瘤分类最近进行了更新,将孤立性纤维肿瘤(SFT)和血管外皮细胞瘤(HPC)重组为一个合并实体。这是第一项基于新 WHO 指南研究 SFT/HPC 患者结局的基于人群的研究。从 1998 年至 2013 年,检索监测、流行病学和最终结果(SEER)数据库,以研究 416 例手术切除病例的总生存率与年龄调整发病率及相关预后因素。计算出年龄调整发病率为每 1000 万人中有 3.77 例,且呈上升趋势。中位生存期为 155 个月,5 年和 10 年生存率分别为 78%和 61%。年龄较小、亚裔/太平洋岛民与白人种族、良性组织学、肿瘤位置、大体全切除(GTR)、GTR 加放疗(RT)与次全切除与生存显著相关。多变量分析显示,年龄较大(HR=1.038,p<0.0001)、幕下位置(HR=2.019,p=0.038)、GTR(HR=0.313,p=0.041)和 GTR+RT(HR=0.215,p=0.008)是独立的预后因素。在 HPC 和交界性/恶性亚组中,与单独 GTR 相比,GTR+RT 显著提高了生存(HR=0.537,p=0.039 和 HR=0.525,p=0.038)。在排除诊断后 3 个月内死亡的患者后,与单独 GTR 相比,GTR+RT 仍与生存的显著提高相关(HR=0.238,p=0.031)(HR=0.280,p=0.054)。GTR+RT 可能是 CNS HPC 和交界性/恶性特征的 SFT/HPC 肿瘤的最佳治疗选择。本研究与现有文献相结合,值得通过前瞻性临床试验进一步研究辅助放疗。