Liver cancer in European children: incidence and survival, 1978-1997. Report from the Automated Childhood Cancer Information System project.

Data on 849 children diagnosed with malignant hepatic tumours (International Classification of Childhood Cancer, Group VII) before the age of 15 years during 1978-1997 in Europe were extracted from the ACCIS database. Age-standardised incidence during 1988-1997 was 1.5 per million overall, 1.2 per million for hepatoblastoma and 0.2 per million for hepatic carcinoma. Over 90% of cases of hepatoblastoma occurred before age 5 years, whereas hepatic carcinoma had a fairly flat age distribution. Both tumours had an incidence in boys of 1.5-1.6 times that in girls. There were no significant time trends in incidence during 1978-1997. Five-year survival from hepatoblastoma diagnosed during 1988-1997 was 63% overall, and ranged from 52% in Eastern Europe to 84% in the North. Survival from hepatic carcinoma was much lower (37%). Between 1978-1982 and 1993-1997, 5-year survival (95% confidence interval (95% CI)) increased from 28% (95% CI 18-39) to 66% (95% CI 55-74) for hepatoblastoma and from 17% (95% CI 6-33) to 50% (95% CI 26-70) for hepatic carcinoma. These increases reflect the impact of advances in treatment of childhood liver cancer at a population level.