Medulloblastoma of the Cerebellopontine Angle in Adults: A Systematic Review of Diagnostic Features, Clinical Management, and Surgical Outcomes

Introduction: Medulloblastoma is a primary central nervous system tumor found most commonly in pediatric patients. MBs are rarely seen in adults and cerebellopontine angle (CPA) medulloblastomas are even rarer, with fewer than 50 cases discussed in the literature. This systematic review provides a thorough characterization of current management strategies employed to treat this uncommon pathology to guide care for future patients.

Methods: Following PRISMA guidelines, a systematic review was conducted including PubMed, EMBASE, Web of Science, and Cochrane databases from inception to June 19, 2024. Studies on adult patients with histologically-confirmed CPA medulloblastomas were included. Data on patient demographics, clinical presentation, management strategies utilized, and patient outcomes were synthesized.

Results: Twenty-seven studies with a total of 42 adult CPA medulloblastoma patients were included. The median age was 32 years (range: 19-56), with a male predominance (64%). Headache (81%), nausea/vomiting (50%), and gait disturbance/ataxia (40%) were the most common presenting symptoms. The predominant histological subtype was the classic variant (57%). Gross total resection was achieved in 59% of cases, with the retrosigmoid approach being the most common surgical technique used (78%). Adjuvant therapy was administered to 98% of patients, with 57% receiving both radiotherapy and chemotherapy. At last follow-up (median: 18 months), the cohort showed a low recurrence rate of 11%. Relatively high survival rates were observed: 96%, 85%, and 85% at 1, 3, and 5 years, respectively. Patients who received adjuvant therapy showed significantly lower recurrence rates and greater overall survival compared to those who did not. We outline the adjuvant therapy approaches utilized for these patients to guide future patient treatment decisions and discussion of therapeutic options.

Conclusion : This review provides comprehensive insights into the typical clinical presentation, treatment options, and patient outcomes in adult CPA medulloblastoma to guide patient care for this rare tumor not frequently encountered clinically.