Clinical profile and outcomes of pediatric ependymoma: a 10-year retrospective analysis from a resource-limited setting.

BACKGROUND

Pediatric ependymomas account for 6-10% of central nervous system tumors in children and are the third most common pediatric brain tumor. However, survival and outcome data from low- and middle-income countries (LMICs), particularly in South Asia, are limited. This study aims to evaluate the clinical profile, treatment approaches, and survival outcomes of pediatric patients with ependymoma in a tertiary care setting in Pakistan.

METHODS

This retrospective cohort study included 48 pediatric and young adult patients (aged 0-21 years) diagnosed with ependymoma and treated at Aga Khan University Hospital, Karachi, from January 2012 to December 2021. Data were collected on demographics, clinical presentation, tumor location, histopathology, treatment modalities, and follow-up. Survival analysis was conducted using SPSS version 23.0, with Kaplan-Meier estimates for overall survival (OS) and progression-free survival (PFS), and comparisons performed using the log-rank test.

RESULTS

Of the 48 patients, 64.6% were male, with a median age of 7.5 years. Presenting symptoms included headache and vomiting (43.75%), limb weakness (25%), back pain (20.83%), and ataxia (18.75%). Tumors were located in the posterior fossa (50%), supratentorial region (29.2%), and spinal cord (20.8%). All patients underwent surgical resection; postoperative complications were noted in a subset. Histopathological subtypes included anaplastic (62.5%), classic (33.3%), and myxopapillary ependymoma (4.2%). Adjuvant radiotherapy was given to 62.5% and chemotherapy to 10.4%. The 10-year overall survival (OS) and progression-free survival (PFS) rates were 79.2% and 66.7%, respectively. Tumor recurrence was observed in 18.8% of cases.

CONCLUSION

Multimodal treatment yielded favorable outcomes despite resource constraints. Early diagnosis and individualized management are crucial for enhancing survival in low- and middle-income country (LMIC) settings.