Abstract BACKGROUND The West Midlands Regional Children’s Tumour Registry is a specialist paediatric cancer registry in the West Midlands region of England, representing 9% of the national population. Our aim was to produce an historical, regional overview of central nervous system (CNS) tumour incidence and survival from 1957 onwards. METHODS Paediatric CNS tumour records included in the Registry from 01/01/57 to 31/12/16 were reviewed. There were 1945 cases: 1066 males and 879 females (M:F ratio 1.2:1) aged 0-14 years, resident in the West Midlands and diagnosed with a malignant, uncertain, or benign tumour. Patients were categorised according to the International Classification of Childhood Cancer, Third Edition (ICCC-3). Age and sex-specific incidence rates per million and directly age standardised incidence rates were calculated. Survival by decade of diagnosis was calculated using Kaplan-Meier survival analysis. RESULTS Incidence rates were broadly comparable to national data. Five-year survival for all CNS tumours combined increased from 38.0% [CI 32.2-43.8%] during 1957-1966 to 79.8% [CI 75.4-83.5] for diagnoses 2007-2016. Five-year survival for patients with ependymoma, astrocytoma and medulloblastoma diagnosed 2007-2016 was 95.7% [CI 72.9-99.4], 86.9% [CI 80.3-91.3] and 68.0% [CI 52.0-79.7] respectively: substantially above equivalent national figures. Five-year survival for groups including ‘Other Gliomas’ and Atypical teratoid/rhabdoid tumour (ATRT) had lower than nationally reported survival rates at 53.3% [CI 37.6-66.8] and 9.1% [0.5-33.3] however, small numbers and wide confidence intervals must be considered. CONCLUSIONS Huge improvements in survival, nationally and in the West Midlands, over the past 60 years are attributable to a greater understanding of tumour biology, intensification of multi-agent chemotherapy and new treatments. Despite these gains, certain diagnoses - most prominently ATRT - remain resistant to cure with five-year survival below 10%. Certain diagnoses showed a notable rise in late mortality, demonstrating the importance of long-term follow-up for detecting late effects and subsequent malignancies.
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