Balasubramanian Kishore, Kharbat Abdurrahman F, Call-Orellana Francisco, Tavakol Sherwin A, Fassina Grace R, Janssen Christopher, Bin Alamer Othman, Zuccato Jeffrey A, Dunn Ian F
College of Medicine, Texas A&M University, 8447 Riverside Parkway, Bryan, TX 77807, USA.
Department of Neurosurgery, University of Oklahoma Health Sciences Center, 1000 N. Lincoln Ave. #4000, Oklahoma City, OK 73104, USA.
Cancers (Basel). 2024 Dec 20;16(24):4242. doi: 10.3390/cancers16244242.
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.
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.
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.
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.
本研究旨在系统回顾文献中关于成人桥小脑角（CPA）髓母细胞瘤（MB）的个体患者数据，描述该罕见实体的患者表现、所采用的治疗策略及肿瘤学结局，以指导未来临床实践。
按照PRISMA指南，通过检索PubMed、EMBASE、科学网和Cochrane数据库（从建库至2024年6月19日）进行系统回顾。纳入关于经组织学确诊且影像学证实位于CPA的成年MB患者的研究。综合临床数据并评估结局预测因素。
纳入27项研究，共42例成年CPA MB患者。中位年龄为32岁（范围：19 - 56岁）。头痛（81%）、颅神经病变（90%）、小脑功能障碍（79%）和恶心/呕吐（50%）是典型的临床表现。主要组织学亚型为经典型。进行了最大安全手术切除，最常用乙状窦后入路，60%的病例实现了全切。大多数患者接受了辅助治疗（93%），通常是放化疗。中位随访18个月后复发率为11%。1年、3年和5年的生存率分别为96%、85%和85%，相对较高。接受辅助治疗的患者复发情况明显较好，总体生存结局更佳。
这些结果支持在影像学特征提示细胞增多的成年年轻CPA肿瘤患者中考虑MB，并采用最大安全切除加术后全脑脊髓照射及化疗的治疗策略来优化治疗这些罕见患者。
