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

A. MacCarthy*, G. J. Draper, Eva Steliarova-Foucher, J. E. Kingston, S. V. Petrovich, O. Budanov, H. Storm, N. Christensen, T. Aareleid, T. Hakulinen, R. Sankila, E. Pukkala, E. Le Gall, I. Tron, B. Lacour, E. Desandes, J. L. Bernard, P. Pillon, J. C. Gentet, F. FreyconC. Berger, L. Remontet, A. Danzon, M. Mercier, J. P. Daurè s, B. Tretarre, F. Ménégoz, A. V. Guizard, M. Velten, A. Buemi, N. Raverdy, M. Sauvage, P. Grosclaude, P. Kaatsch, B. Eisinger, R. Stabenow, D. Schuler, Z. Jakab, G. Borgulya, L. Tryggvadottir, J. G. Jonasson, K. Bjarnadottir, H. Comber, F. Dwane, C. Magnani, G. Pastore, F. Pannelli, C. Pascucci, S. Ferretti, E. Conti, V. Ramazzotti, M. C. Cercato, M. Vercelli, A. Puppo, P. Crosignani, G. Tagliabue, A. Tittarelli, V. De Lisi, P. Sgargi, R. Tumino, M. Budroni, D. Piras, E. Paci, E. Crocetti, F. La Rosa, F. Stracci, P. Zambon, S. Guzzinati, M. Dalmas, J. W.W. Coebergh, J. van Dijck, A. Wit, F. Langmark, A. Johansen, A. Andersen, I. Plesko, M. Primic Z? akelj, V. Pompe-Kirn, R. Peris-Bonet, B. Giner, E. Almar Marques, A. Mateos Ramos, J. Ramon Quiros Garcia, A. Can˜ ada Martínez, I. Izarzugaza, A. Alemán Herrera, P. Viladiu, R. Marcos, A. Izquierdo, C. Martínez Garcia, A. Obrador, I. Garau, E. Ardanaz, J. Borrà s, J. Galceran, J. de la Bá rcena Guallar, M. C. Martos Jiménez, G. Jundt, C. Bouchardy, M. Usel, J. Allemann, H. Frick, T. Fisch, S. Ess, F. Joris, D. de Weck, S. Yalcin Eser, C. A. Stiller, M. F.G. Murphy, A. Gavin, C. Fox, W. Hamill, R. Middleton, D. Brewster, L. Bhatti, A. McDonald

*Corresponding author for this work

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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.

Original languageEnglish
Pages (from-to)2092-2102
Number of pages11
JournalEuropean Journal of Cancer
Issue number13
Publication statusPublished - 1 Sept 2006

Bibliographical note

Funding Information:
The ACCIS project was funded by the European Commission from the Europe Against Cancer programme (contracts SI2.126875, SI2.321970 and SPC.2002303), jointly with the International Agency for Research on Cancer. Data analyses were partly funded by the French Ligue Nationale contre le Cancer, Comité du Rhône . This work was undertaken with the help of a research grant for epidemiological studies of retinoblastoma from the Childhood Eye Cancer Trust (CHECT). We are grateful to colleagues at the Childhood Cancer Research Group for help with this study. The Chidhood Cancer Research Group receives funding from the Department of Health and the Scottish Ministers. The funding agencies had no role in the design, conduct, reporting, or decision to publish the study. The views expressed here are those of the authors and not necessarily those of the Department of Health and the Scottish Ministers.

Other keywords

  • Cancer
  • Childhood
  • Europe
  • Incidence
  • Registry
  • Retinoblastoma
  • Survival
  • Trends


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