Variation in survival of children with central nervous system (CNS) malignancies diagnosed in Europe between 1978 and 1992: The EUROCARE study

EUROCARE is a population-based survival study including data from European Cancer Registries. The present paper analyses survival after a malignant neoplasm of the central nervous system (CNS) in childhood (aged 0-14 years at diagnosis). The database includes 6130 cases from 34 population-based registries in 17 countries: 1558 were primitive neuroectodermal tumours (PNET) and 4087 astrocytoma, ependymoma or other gliomas: these morphologies were grouped in the analyses in order to reduce the diagnostic variability among the registries. 87% of cases were microscopically diagnosed (range among registries 71-100%) and losses to follow-up were limited to 2% (range 0-14%). Actuarial analyses indicate that the European (weighted) average of 5 years cumulative survival for cases diagnosed in 1978-1989 was 53% (95% confidence interval (CI) 49-57) for CNS neoplasms, 44% (95% CI 37-50) for PNET and 60% (95% CI 55-65) for the glioma-related types. Analysis of the sub-set of cases diagnosed in 1985-1989 revealed better results: cumulative survival at 5 years was 61% (95% CI: 55-65) for all CNS neoplasms; 48% (95% CI 41-56) for PNET and 68% (95% CI 62-73) for glioma-related types. Compared with older children, infants showed poorer prognosis: in 1978-1989 the 5-year survival rate was 33% (95% CI 23-45) and in 1985-1989 it was 46% (95% CI 34-59). Variability among countries was very large, with 5-year survival for CNS tumours diagnosed in 1985-1989 ranging from 28% in Estonia (95% CI 17-43) to 73% Sweden (95% CI 59-83) and 75% in Iceland (95% CI 35-95) and 73% in Finland (95% CI 66-79). Time trends were studied in a multivariate analysis observing a reduction in the risk of death in periods of diagnosis 1982-1985 (hazard ratio (HR) = 0.85; 95% CI 0.78-0.93) and 1986-1989 (HR = 0.70; 95% CI 0.64-0.77) compared with 1978-1981. The analysis were extended to 1990-1992 for the countries whose registries provided data for that period did not indicate any further progress. Results of this study confirm the large variability in European countries and indicate a positive trend in the survival probability for cases diagnosed in the 1980s.