Abstract
INTRODUCTION: Intracranial ependymoma, the 3(rd)-most common pediatric brain tumor, has poor long-term survival. Our aim was to study the risk-factors for progression/relapse in a new cancer hospital in eastern India. METHODS: Retrospectively extracted data from case records between 2012–17 were analyzed. RESULTS: Eighteen children were enrolled (median age: 39.5 months, males: 55%). Median symptom-diagnosis interval was 8 weeks. Symptoms included headache (8; 44%), vomiting (10; 55%), unsteadiness (8; 44%), seizures/altered sensorium (4; 22%). Location was posterior fossa (12; 67%), or supra-tentorial (6; 33%). Spinal/leptomeningeal spread was documented in 4/14 (28.5%). Complete excision was performed in 4/16 (25%); second-look surgery was feasible in one. Histopathology was anaplastic (14; 78%) or grade II (4; 22%). Sixteen children received adjuvant treatment: 11 (69%) radiotherapy alone, 5 (31%) additional chemotherapy. One each, refused treatment (died), and chose homeopathy (alive). Median survival was 30 months. 3-year progression-free survival was 39.5 ± 1.7%. Patterns of failure were local in 5/6, and combined (local and metastatic) in one. One child is on salvage treatment, rest died. Age, gender, symptom-diagnosis interval, location, subtotal resection, anaplastic histology, leptomeningeal dissemination and nature of adjuvant therapy were not predictive of failure (p>0.05). Two/4 children who had complete resection relapsed 30 and 14 months after completing treatment. There was no treatment-related mortality. CONCLUSION: Pediatric ependymoma remains a difficult disease to treat, with failures, predominantly local, occurring despite complete resection and adjuvant therapy, several months after completing treatment. Novel treatment strategies based on molecular classification, and regular surveillance for detecting relapse early, may improve outcome.