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Abstract：
Background Despite advances in multimodal oncologic therapies and molecular genetics, overall survival (OS) in patients with high-grade astrocytomas remains poor. We present an illustrative case and systematic review of rare, predominantly extra-axial World Health Organization (WHO) grade 4 astrocytomas located within the cerebellopontine angle (CPA) and explore the impact of anatomic location on diagnosis, management, and outcomes. Methods A systematic review of adult patients with predominantly extra-axial WHO grade 4 CPA astrocytomas was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines through December 2022. Results Eighteen articles were included comprising 21 astrocytomas: 13 exophytic tumors arising from the cerebellopontine parenchyma and 8 tumors originating from a cranial nerve root entry zone. The median OS was 15 months with one-third of cases demonstrating delayed diagnosis. Gross total resection, molecular genetic profiling, and use of ancillary treatment were low. We report the only patient with an integrated isocitrate dehydrogenase 1 (IDH-1) mutant diagnosis, who, after subtotal resection and chemoradiation, remains alive at 40 months without progression. Conclusion The deep conical-shaped corridor and abundance of eloquent tissue of the CPA significantly limits both surgical resection and utility of device-based therapies in this region. Prompt diagnosis, molecular characterization, and systemic therapeutic advances serve as the predominant means to optimize survival for patients with rare skull base astrocytomas.
摘要：
背景尽管在多模式肿瘤治疗和分子遗传学方面取得了进展，高级别星形细胞瘤患者的总生存期(OS)仍然较差.我们提出了一个说明性的案例和系统的回顾，主要位于桥脑小脑角（CPA）内的4级星形细胞瘤，并探讨解剖位置对诊断的影响，管理,和结果。方法根据系统评价和荟萃分析（PRISMA）指南的首选报告项目，到2022年12月，对主要为轴外型WHO4级CPA星形细胞瘤的成年患者进行系统评价。结果共纳入18篇文献，包括21例星形细胞瘤：13例由桥小脑实质引起的外生性肿瘤和8例由颅神经根进入区引起的肿瘤。中位OS为15个月，三分之一的病例显示延迟诊断。总切除,分子遗传学分析，辅助治疗的使用率较低。我们报道了唯一一个具有整合的异柠檬酸脱氢酶1（IDH-1）突变诊断的患者，谁,次全切除和放化疗后，在40个月时仍然存活，没有进展。结论CPA的深圆锥形走廊和丰富的雄辩组织极大地限制了该区域的手术切除和基于设备的治疗的实用性。及时诊断,分子表征，系统性治疗进展是优化罕见颅底星形细胞瘤患者生存的主要手段。
