OBJECTIVE The rarity of intracranial extraventricular neurocytomas (EVNs) has precluded accurate definition of its surgical characteristics to date. The authors present the first survival analysis of this unique entity that aims to clarify tumor characteristics, surgical outcomes, and efficacy of postoperative adjuvant therapy. METHODS A systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Searches of the MEDLINE, Embase, Scopus, Google Scholar, and Cochrane Database of Systematic Reviews databases were performed from inception to date. Primary outcomes were progression-free survival (PFS) and overall survival (OS). Prognostic variables were age, sex, tumor consistency, extent of resection, and receipt of postoperative adjuvant therapy. Survival data were analyzed using Kaplan-Meier survival curves and the log-rank test to compare dichotomized cohorts. Multivariate Cox regression models were constructed, interrogated with Schoenfeld residuals, and subsequently utilized to identify independent prognostic factors. Risk of bias was assessed with the Mayo Clinic instrument. RESULTS Five hundred fourteen articles were initially retrieved, which was distilled to 10 included articles consisting of 101 cases of intracranial EVNs. The 5-year OS rate was 90.4% (95% CI 81.8%-99.8%) and the PFS rate was 48.6% (95% CI 34.46%-68.8%). The median PFS was 60 months. Patients younger than 50 years of age experienced superior OS (p = 0.03) and PFS (p < 0.01). Gross-total resection (GTR) was superior to subtotal resection (STR) in reducing mortality (p < 0.01). Adjuvant therapy following either STR or GTR did not significantly improve survival. CONCLUSIONS Intracranial EVNs are rare tumors that portend a poorer prognosis than central neurocytomas, despite both being WHO grade 2 tumors. Complete surgical extirpation is the cornerstone of management. There is no clearly established role for adjuvant postoperative therapy, but each case should be managed on an individual basis.

中文翻译：

---

颅内室外神经细胞瘤的生存结果：系统评价和个体患者数据荟萃分析。

目的 迄今为止，颅内室外神经细胞瘤（EVN）的罕见性阻碍了其手术特征的准确定义。作者对这一独特实体进行了首次生存分析，旨在阐明肿瘤特征、手术结果和术后辅助治疗的疗效。方法 根据系统评价和荟萃分析指南的首选报告项目进行系统评价。从建立至今，均对 MEDLINE、Embase、Scopus、Google Scholar 和 Cochrane Database of Systematic Reviews 数据库进行检索。主要结局是无进展生存期（PFS）和总生存期（OS）。预后变量包括年龄、性别、肿瘤一致性、切除范围以及接受术后辅助治疗。使用 Kaplan-Meier 生存曲线和对数秩检验分析生存数据，以比较二分队列。构建了多变量 Cox 回归模型，用 Schoenfeld 残差进行询问，随后用于识别独立的预后因素。使用梅奥诊所仪器评估偏倚风险。结果 初步检索到514篇文章，最终收录文章10篇，共101例颅内EVN。5年OS率为90.4%（95% CI 81.8%-99.8%），PFS率为48.6%（95% CI 34.46%-68.8%）。中位 PFS 为 60 个月。50 岁以下的患者具有较高的 OS (p = 0.03) 和 PFS (p < 0.01)。在降低死亡率方面，大体全切除 (GTR) 优于次全切除 (STR) (p < 0.01)。STR 或 GTR 后的辅助治疗并未显着提高生存率。结论 颅内 EVN 是一种罕见的肿瘤，尽管都是 WHO 2 级肿瘤，但其预后比中枢神经细胞瘤更差。彻底的手术切除是治疗的基石。术后辅助治疗的作用尚无明确规定，但应根据每个病例进行个体化处理。