Childhood central nervous system tumours - incidence and survival in Europe (1978-1997): Report from the Automated Childhood Cancer Information System project.

Abstract This paper describes the incidence and survival of childhood central nervous system (CNS) tumours in Europe for the period 1978-1997. A total of 19,531 cases, aged 0-14 years, from the ACCIS database were analysed by five regions: the British Isles, East, North, South, and West. Overall age-standardised incidence rate (ASR) of CNS tumours in Europe (1988-1997) was 29.9 per million, with the highest rates in the North. Astrocytoma (ASR=11.8), primitive neuroectodermal tumours (PNET) (ASR=6.5) and ependymoma (ASR=3.4) were the most frequent types. Incidence increased significantly during 1978-1997, on average by 1.7% per year. Diagnostic methods may partially explain incidence rates and trends, although a role of variations in risk factors cannot be excluded. Overall 5-year survival was 64% and varied between 72% in the North and 53% in the East. PNET had the poorest prognosis (49%) and astrocytoma the best (75%). Survival has improved by 29% since late 1970s. The positive trends were seen in all regions, although the interregional differences persisted, as a reflection of the different healthcare systems.