ID #887 Medulloblastoma: Experience from a Tertiary Care Centre of Pakistan

Medulloblastoma (MB) is the most common malignant pediatric brain tumor representing approximately 20% of all primary central nervous system (CNS) tumors. Seventy percent occurs in children under 10 years of age and males are affected approximately 1.7 times more than females.

Methods

Children younger than 18 years diagnosed with medulloblastoma, at Aga Khan University Hospital’s (AKUH) paediatric oncology in-patient and out-patient setting from 2013 to 2025, were included. Data was collected retrospectively using patient files and the analysis was done using R software.

Results

Sixty-seven patients with MB were included. The median age at diagnosis was 8 years (IQR 4–12.5), with a male predominance of 71.6%. Headache (79.1%) and vomiting (71.6%) were the most common presenting symptoms, followed by ataxia and cranial nerve palsy in 34.3% and 23.9% patients, respectively. The median duration of symptoms was 1 month (IQR: 0.58 - 1.0 months). Gross total resection (GTR) was achieved in 51 patients (76%), and 16 patients (24%) had subtotal resection (STR). According to clinical risk group 52% were high risk, and 47.8% had average risk. In our cohort 34% had metastatic disease. Classic/NOS MB was identified in 66% patients, 31% with desmoplastic nodular and 2% had large cell anaplastic histology. Eight patients were p53 positive. Molecular subgroup by immunohistochemistry and next generation sequencing; WNT 7.5%, SHH 19%, non WNT/ non SHH 62.5%, and non-done/inconclusive 10.5%. 26.5% received high risk chemotherapy while 73.5% patients received average risk chemotherapy. Ten patients (24.4%) had relapsed There were 10.4% lost to follow up and 28.3% (19) patients were referred to another hospital. The estimated 5-year overall survival (OS) for the cohort was 76%.

Conclusion

Our institutional experience highlights the clinical profile and management of patients incorporating multidisciplinary teams and adherence to treatment protocols. Strengthening multidisciplinary collaboration, providing access to advanced neuro-oncology services, by addressing the socioeconomic barriers are essential for the improved outcomes for medulloblastoma patients in Pakistan.