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

This population-based study is based on 5802 cases of soft tissue sarcomas (STS) in children aged 0-14 years extracted from the database of the Automated Childhood Cancer Information System (ACCIS) and registered in population-based cancer registries in Europe for the period 1978-1997. STS represent almost 8% of neoplasms in children, almost half of whom are less than 5 years at diagnosis. Rhabdomyosarcoma is the most frequent childhood STS (50%). During 1988-1997 the age-standardised incidence of STS in Europe was 9.1 per million children, lowest in the West and East and highest in the North. The incidence of STS increased almost 2% per year over the period 1978-1997, attributable mostly to increase in genito-urinary rhabdomyosarcoma. Prognosis of children with STS was related to age and site of tumour. Five-year survival of children with STS increased from 46% in 1978-1977 to 66% in 1993-1997, reaching 74% in the North for those diagnosed in 1993-1997. This improvement is ascribed to therapy advances.