Abstract
Based on 2283 cases of retinoblastoma diagnosed in children aged 0–14 years, incidence
and survival in Europe during the period 1978–1997 are described. Data were provided
to the Automated Childhood Cancer Information System (ACCIS) from 60 paediatric and
general cancer registries. During 1988–1997, the cumulative incidence of retinoblastoma
in the ACCIS regions was found to be between 44.2 and 67.9 per million births. The
highest incidence was seen in the first year of life. The age-standardised (World
standard) incidence rate for the age-range 0–14 years was 4.1 per million. Approximately
one-third of cases had bilateral tumours. Overall incidence increased over the period
1978–1997 by 1% per year, as derived from a model adjusted for sex, age group and
type of registry (general or paediatric). The 5-year survival rate improved from 89%
to 95% during the period covered by the study. This improvement was seen in both unilateral
and bilateral cases but was significant only for the unilateral tumours. Survival
was lower in the East region, although smaller differences were also observed between
the other four regions (British Isles, North, South and West). Availability and quality
of registration data on retinoblastoma need to be improved for effective evaluation
of incidence and survival.
Keywords
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© 2006 Elsevier Ltd. Published by Elsevier Inc. All rights reserved.
