脑室内胶质母细胞瘤（IVGBM）是中枢神经系统内的罕见肿瘤，由于其位于脑室系统内，其诊断和治疗面临着独特的挑战。尽管这些肿瘤很罕见，但需要进行全面研究，以完善诊断方法并优化治疗策略。使用 PubMed、Scopus、Web of Science 和 Google Scholar 数据库进行了系统评价，以确定截至 2024 年 1 月发表的相关文献。纳入标准包括研究英语，重点介绍 IGBM 的临床特征、放射学特征、病理学和治疗。数据合成和分析遵循系统评价和荟萃分析的首选报告项目 (PRISMA) 指南。24 篇文章符合纳入标准，其中包括 47 名 IGBM 患者。中位年龄为 47 岁，男性占多数（男性 32 名，女性 15 名）。常见症状包括颅内压升高和癫痫发作。肿瘤主要影响侧脑室（体部和脑三角）。手术切除（次全切除或总切除）是主要治疗方法，术后进行辅助治疗（放疗、化疗）。IVGBM 由于其心室位置而提出了独特的诊断和治疗挑战。目前的治疗主要包括手术切除，然后进行辅助治疗，但结果仍令人担忧。需要进一步的研究来加强对这种罕见的胶质母细胞瘤亚型的理解和管理。

Intraventricular glioblastomas (IVGBMs) are rare tumors within the central nervous system characterized by unique challenges in diagnosis and management due to their location within the ventricular system. Despite their rarity, these tumors necessitate comprehensive study to refine diagnostic approaches and optimize therapeutic strategies.A systematic review was conducted using PubMed, Scopus, Web of Science, and Google Scholar databases to identify relevant literature published up to January 2024. Inclusion criteria encompassed studies in English focusing on clinical characteristics, radiological features, pathology, and treatment of IVGBM. Data synthesis and analysis followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.Twenty-four articles met the inclusion criteria, comprising 47 patients with IVGBM. The median age was 47 years, with a male predominance (32 males, 15 females). Common symptoms included increased intracranial pressure and seizures. Tumors predominantly affected the lateral ventricles (body and trigone). Surgical resection (subtotal or gross total) was the primary treatment approach, with adjuvant therapies (radiotherapy, chemotherapy) administered postoperatively.IVGBM present distinct diagnostic and therapeutic challenges due to their ventricular location. Current treatments primarily involve surgical resection followed by adjuvant therapies, though outcomes remain guarded. Further research is needed to enhance understanding and management of this rare glioblastoma subset.