Neuroblastoma incidence and survival in European children (1978-1997): report from the Automated Childhood Cancer Information System project.

The Automated Childhood Cancer Information System (ACCIS) collects and presents data on childhood cancer in Europe. This report describes trends (1978-1997) and geographical differences (1988-1997) in incidence and survival for 6202 children with neuroblastoma from 59 registries in 19 countries, grouped into five regions (British Isles, West, East, North, and South). The age-standardised incidence rate (ASR) of neuroblastoma in Europe in 1988-1997 was 10.9 cases per million children, being highest in infants (52.6). The ASR of neuroblastoma increased in Europe from 8.4 in 1978-1982 to 11.6 in 1993-1997, mostly due to an increase in infants (from 35.4 to 57.8). Overall 5-year survival was 59%, ranging from 47% (East) to 67% (West). It improved markedly from 37% in 1978-1982 to 66% in 1993-1997, especially in infants. A certain amount of overdiagnosis in children under 2 years of age may explain the increased incidence rates and partially the increase in survival. Survival of older children (aged 2-14 years), which is likely to be largely affected by therapy, has also improved from 21% to 45%.