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

Abstract

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.