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.