Objective: The aim of this study was to systematically review the existing individual patient data in the literature on adult cerebellopontine angle (CPA) medulloblastoma (MB) and characterize the patient presentation, management strategies used, and oncological outcomes of this rare entity to guide future clinical practice. Methods: Following PRISMA guidelines, a systematic review was conducted by searching PubMed, EMBASE, Web of Science, and Cochrane databases from inception to 19 June 2024. Studies regarding adult patients with histologically confirmed MB radiographically confirmed to be located in the CPA were included. Clinical data were synthesized, and predictors of outcomes were evaluated. Results: Twenty-seven studies with 42 adult CPAMB patients were included. The median age was 32 years (range: 19–56). Headaches (81%), cranial neuropathy (90%), cerebellar dysfunction (79%), and nausea/vomiting (50%) were typical presenting features. The predominant histological subtype was the classic variant. Maximal safe surgical resection was performed, most commonly using a retrosigmoid approach, and 60% of cases received a gross total resection. Most patients received adjuvant treatment (93%), typically chemoradiotherapy. The recurrence rate was 11% after a median of 18 months of follow-up. Relatively high survival rates of 96%, 85%, and 85% were observed at 1, 3, and 5 years, respectively. Patients who received adjuvant therapy had significantly better recurrence and greater overall survival outcomes. Conclusions: These results support the consideration of MB in young adult patients presenting with CPA tumors with radiographical features suggestive of hypercellularity and the utilization of a management strategy of maximal safe resection plus post-operative craniospinal irradiation along with chemotherapy to optimally treat these rare patients.
目的：本研究旨在系统回顾现有文献中关于成人桥小脑角区髓母细胞瘤的个体患者数据，并描述该罕见疾病的临床表现、治疗策略及肿瘤学预后，以指导未来临床实践。方法：遵循PRISMA指南，通过检索PubMed、EMBASE、Web of Science和Cochrane数据库自建库至2024年6月19日的文献进行系统综述。纳入经组织学确诊且影像学证实位于桥小脑角区的成人髓母细胞瘤病例研究。对临床数据进行整合分析，并评估预后影响因素。结果：共纳入27项研究，涉及42例成人桥小脑角区髓母细胞瘤患者。中位年龄32岁（范围：19-56岁）。典型临床表现包括头痛（81%）、颅神经功能障碍（90%）、小脑功能失调（79%）及恶心呕吐（50%）。组织学亚型以经典型为主。主要采用乙状窦后入路进行最大安全范围手术切除，60%病例实现肿瘤全切除。绝大多数患者（93%）接受辅助治疗，主要为放化疗联合方案。中位随访18个月后复发率为11%。1年、3年和5年生存率分别为96%、85%和85%。接受辅助治疗的患者在复发控制和总生存期方面均有显著改善。结论：研究结果支持对具有高细胞密度影像学特征的年轻成人桥小脑角区肿瘤患者应考虑髓母细胞瘤诊断，并建议采用最大安全范围切除联合术后全脑全脊髓放疗及化疗的综合治疗策略，以实现对该罕见患者群体的最佳治疗。
肿瘤(癌症)患者之家