Department of Neurosurgery, Stanford University School of Medicine, Stanford, CA, 94305, USA.
J Neurooncol. 2018 Mar;137(1):11-22. doi: 10.1007/s11060-017-2697-0. Epub 2017 Dec 4.
Hemangioblastomas are rare, benign, vascular tumors of the central nervous system (CNS), often associated with von-hippel lindau (VHL) disease. Current therapeutic options include microsurgical resection or stereotactic radiosurgery (SRS). With no randomized controlled studies and minimal data beyond single-institution reviews, the optimal management approach for patients with CNS hemangioblastomas is unclear. We completed a Pubmed/SCOPUS literature search from January 1990 to January 2017 for eligible studies on SRS for CNS hemangioblastomas. Relevant articles were identified and reviewed in accordance to the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) guidelines. 26 studies met eligibility criteria for qualitative synthesis, representing 596 subjects and 1535 tumors. The Gamma Knife was the most published SRS method for CNS hemangioblastomas. After critical study appraisal for intra-study bias, 14 studies were used for quantitative meta-analysis of 5-year progression free survival (PFS). The pooled 5-year PFS across all eligible studies was 88.4%. No difference was observed between spine versus intracranial studies. Individual patient data (IPD) was extracted from 14 studies, representing 322 tumors. Univariate analysis of IPD revealed that VHL patients were younger, and had smaller tumors compared to those with sporadic disease. Adverse events were associated with increasing marginal dose, independent of tumor volume. VHL status, sex, radiosurgical method, tumor location, and tumor volume were not found to be significantly associated with tumor progression. Multiple studies show excellent tumor control at 5-year follow up, however, the long-term efficacy of SRS for CNS hemangioblastomas still needs to be investigated, and the studies exploring the role of SRS for early treatment of asymptomatic lesions is wanting.
血管母细胞瘤是一种罕见的中枢神经系统（CNS）良性血管肿瘤，常与 von-Hippel-Lindau（VHL）病相关。目前的治疗选择包括显微手术切除或立体定向放射外科手术（SRS）。由于没有随机对照研究，且单一机构回顾性研究的数据有限，因此，CNS 血管母细胞瘤患者的最佳治疗方法尚不清楚。我们对 1990 年 1 月至 2017 年 1 月的 Pubmed/SCOPUS 文献进行了检索，以查找适合 CNS 血管母细胞瘤 SRS 的研究。根据系统评价和荟萃分析的首选报告项目（PRISMA）指南，确定并审查了相关文章。有 26 项研究符合定性综合的入选标准，代表 596 名患者和 1535 个肿瘤。伽玛刀是发表最多的 CNS 血管母细胞瘤 SRS 方法。经过对研究内偏倚的严格评估，有 14 项研究对 5 年无进展生存率（PFS）进行了定量荟萃分析。所有合格研究的 5 年 PFS 汇总率为 88.4%。脊柱与颅内研究之间无差异。从 14 项研究中提取了 14 项研究的个体患者数据（IPD），代表 322 个肿瘤。IPD 的单变量分析显示，VHL 患者比散发性疾病患者更年轻，肿瘤更小。与肿瘤体积无关，不良事件与边缘剂量增加相关。VHL 状态、性别、放射外科方法、肿瘤位置和肿瘤体积与肿瘤进展无显著相关性。多项研究表明，5 年随访时肿瘤控制良好，但 SRS 治疗 CNS 血管母细胞瘤的长期疗效仍需进一步研究，且探索 SRS 在早期治疗无症状病变中作用的研究也较少。
