Hodgkin’s disease incidence and survival in European children and adolescents (1978–1997): Report from the Automated Cancer Information System project


      This paper reports the geographical patterns and time trends of incidence and survival of Hodgkin’s disease (HD) in children and adolescents in Europe over the period 1978–1997. Data on 4230 HD cases were gathered from 62 paediatric or general cancer registries in 19 European countries by the Automated Cancer Information System (ACCIS). European annual incidence rates in 1988–1997 were estimated at 5.8 per million in children (world age-standardised) and at 29.7 per million in adolescents, with higher rates in the East and South. Incidence rates increased steeply with age, while the male predominance, marked for the youngest children, vanished in the highest age groups. Over the period 1978–1997 incidence rates increased in age groups 10–14 years (+1% per year) and 15–19 years (+3.5% per year), mainly due to the nodular sclerosis subtype. Age and sex distribution of cases remained unchanged with time. The overall 5-year survival rate was higher in children (93%, 95% confidence interval (CI) 92–94) than in adolescents (89% (95% CI 87–91)) for the period 1988–1997. Five-year survival increased significantly in all regions from 87% to 93% in children and from 80% to 88% in adolescents between 1978–1982 and 1993–1997. In future, detailed documentation of cases in the cancer registries with respect to standardised diagnostic subtypes, stage of extension, and treatments, will help to refine interpretation of international and temporal variations in incidence and survival.


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        • MacMahon B.
        • Cole P.
        • Newell G.R.
        Hodgkin’s disease: one entity or two?.
        Lancet. 1971; 1: 240-241
        • Glaser S.L.
        • Lin R.J.
        • Stewart S.L.
        • et al.
        Epstein-Barr virus-associated Hodgkin’s disease: epidemiologic characteristics in international data.
        Int J Cancer. 1997; 70: 375-382
        • Armstrong A.A.
        • Alexander F.E.
        • Cartwright R.
        • et al.
        Epstein-Barr virus and Hodgkin’s disease: further evidence for the three disease hypothesis.
        Leukemia. 1998; 12: 1272-1276
        • Weiss L.M.
        • Movahed L.A.
        • Warnke R.A.
        • Sklar J.
        Detection of Epstein-Barr viral genomes in Reed-Sternberg cells of Hodgkin’s disease.
        N Engl J Med. 1989; 320: 502-506
        • Jarrett A.F.
        • Armstrong A.A.
        • Alexander E.
        Epidemiology of EBV and Hodgkin’s lymphoma.
        Ann Oncol. 1996; 7: 5-10
        • Gallagher A.
        • Perry J.
        • Freeland J.
        • et al.
        Hodgkin lymphoma and Epstein-Barr virus (EBV): no evidence to support hit-and-run mechanism in cases classified as non-EBV-associated.
        Int J Cancer. 2003; 104: 624-630
        • Gallagher A.
        • Perry J.
        • Shield L.
        • Freeland J.
        • MacKenzie J.
        • Jarrett R.F.
        Viruses and Hodgkin disease: no evidence of novel herpesviruses in non-EBV-associated lesions.
        Int J Cancer. 2002; 101: 259-264
        • Mack T.M.
        • Cozen W.
        • Shibata D.K.
        • et al.
        Concordance for Hodgkin’s disease in identical twins suggesting genetic susceptibility to the young-adult form of the disease.
        N Engl J Med. 1995; 332: 413-418
        • Goldin L.R.
        • Pfeiffer R.M.
        • Gridley G.
        • et al.
        Familial aggregation of Hodgkin lymphoma and related tumors.
        Cancer. 2004; 100: 1902-1908
        • Stiller C.A.
        What causes Hodgkin’s disease in children?.
        Eur J Cancer. 1998; 34: 523-528
        • Cozen W.
        • Gill P.S.
        • Ingles S.A.
        • Masood R.
        • Martinez-Maza O.
        • et al.
        IL-6 levels and genotype are associated with risk of young adult Hodgkin lymphoma.
        Blood. 2004; 103: 3216-3221
        • Diepstra A.
        • Niens M.
        • te Meerman G.J.
        • Poppema S.
        • van den Berg A.
        Genetic susceptibility to Hodgkin’s lymphoma associated with the human leukocyte antigen region.
        Eur J Haematol Suppl. 2005; : 34-41
        • Percy C.L.
        • Smith M.A.
        • et al.
        Lymphomas and reticuloendothelial neoplasms.
        in: Ries L.A.G. Smith M.A. Gurney J.G. Cancer incidence and survival among children and adolescents: United States – SEER Program 1975–1995, National Cancer Institute, SEER Program. NIH pub. no. 99-4649. National Cancer Institute, SEER Program, Bethesda, MD1999
        • Pastore G.
        • Magnani C.
        • Verdecchia A.
        • Pession A.
        • Viscomi S.
        • Coebergh J.W.
        Survival of childhood lymphomas in Europe, 1978–1992: a report from the EUROCARE study.
        Eur J Cancer. 2001; 37: 703-710
        • Gatta G.
        • Capocaccia R.
        • Stiller C.
        • et al.
        Childhood cancer survival trends in Europe: a EUROCARE Working Group study.
        J Clin Oncol. 2005; 23: 3742-3751
        • Steliarova-Foucher E.
        • Stiller C.
        • Kaatsch P.
        • et al.
        Geographical patterns and time trends of cancer incidence and survival among children and adolescents in Europe since the 1970s (the ACCISproject): an epidemiological study.
        Lancet. 2004; 364: 2097-2105
        • Stiller C.A.
        • Parkin D.M.
        International variations in the incidence of childhood lymphomas.
        Paediatr Perinat Epidemiol. 1990; 4: 303-324
        • Kramárová E.
        • Stiller C.A.
        The international classification of childhood cancer.
        Int J Cancer. 1996; 68: 759-765
        • Percy C.
        • Van Holten V.
        • Muir C.
        International classification of diseases for oncology.
        second ed. World Health Organization, Geneva1990
        • Kalbfleisch J.D.
        • Prentice R.L.
        The statistical analysis of failure time data.
        John Wiley & Sons, New York1980
      1. Sharp L, Cotton S, Little J. Descriptive epidemiology. In: IARC publication No. 149. Epidemiology of childhood cancer. Lyon; 1999, p. 10–66.

      2. Parkin DM, Kramárová E, Draper GJ, et al., editors. International incidence of childhood cancer. Volume 2 IARC scientific publication no.144. Lyon: IARC; 1998.

        • McNally R.J.
        • Kelsey A.M.
        • Cairns D.P.
        • Taylor G.M.
        • Eden O.B.
        • Birch J.M.
        Temporal increases in the incidence of childhood solid tumors seen in Northwest England (1954–1998) are likely to be real.
        Cancer. 2001; 92: 1967-1976
        • Birch J.M.
        • Alston R.D.
        • Kelsey A.M.
        • Quinn M.J.
        • Babb P.
        • McNally R.J.
        Classification and incidence of cancers in adolescents and young adults in England 1979–1997.
        Br J Cancer. 2002; 87: 1267-1274
        • Gatta G.
        • Capocaccia R.
        • Coleman M.P.
        • Ries L.A.
        • Berrino F.
        Childhood cancer survival in Europe and the United States.
        Cancer. 2002; 95: 1767-1772
        • Shankar A.G.
        • Ashley S.
        • Radford M.
        • Barrett A.
        • Wright D.
        • Pinkerton C.R.
        Does histology influence outcome in childhood Hodgkin’s disease? Results from the United Kingdom Children’s Cancer Study Group.
        J Clin Oncol. 1997; 15: 2622-2630
        • Smith R.S.
        • Chen Q.
        • Hudson M.M.
        • et al.
        Prognostic factors for children with Hodgkin’s disease treated with combined-modality therapy.
        J Clin Oncol. 2003; 21: 2026-2033
        • Bleyer W.A.
        Cancer in older adolescents and young adults: epidemiology, diagnosis, treatment, survival, and importance of clinical trials.
        Med Pediatr Oncol. 2002; 38: 1-10
        • Stiller C.
        Epidemiology of cancer in adolescents.
        Med Pediatr Oncol. 2002; 39: 149-155
        • Boissel N.
        • Auclerc M.F.
        • Lhéritier V.
        • et al.
        Should adolescents with acute lymphoblastic leukemia be treated as old children or young adults? Comparison of the French FRALLE-93 and LALA-94 trials.
        J Clin Oncol. 2003; 21: 774-780
        • Brenner H.
        Up-to-date survival curves of children with cancer by period analysis.
        Br J Cancer. 2003; 88: 1693-1697