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.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to European Journal of Cancer
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


      1. Parkin D.M. Kramárová E. Draper G.J. International incidence of childhood cancer: volume 2. IARC, Lyon1998
      2. Hamilton S.R. Aaltonen L. Pathology and genetics of tumours of the digestive system. IARC Press, Lyon2000
        • DeBaun M.R.
        • Tucker M.A.
        Risk of cancer during the first four years of life in children from The Beckwith-Wiedemann Syndrome Registry.
        J Pediatr. 1998; 132: 398-400
        • Garber J.E.
        • Li F.P.
        • Kingston J.E.
        • et al.
        Hepatoblastoma and familial adenomatous polyposis.
        J Natl Cancer Inst. 1988; 80: 1626-1628
        • Ikeda H.
        • Matsuyama S.
        • Tanimura M.
        Association between hepatoblastoma and very low birth weight: a trend or a chance.
        J Pediatr. 1997; 130: 557-560
        • Reynolds P.
        • Urayama K.Y.
        • Von Behren J.
        • Feusner J.
        Birth characteristics and hepatoblastoma risk in young children.
        Cancer. 2004; 100: 1070-1076
        • Ansell P.
        • Mitchell C.D.
        • Roman E.
        • et al.
        Relationships between perinatal and maternal characteristics and hepatoblastoma: a report from the UKCCS.
        Eur J Cancer. 2005; 41: 741-748
        • Chang M.-H.
        • Chen C.-J.
        • Lai M.-S.
        • et al.
        Universal hepatitis B vaccination in Taiwan and the incidence of hepatocellular carcinoma in children.
        N Eng J Med. 1997; 336: 1855-1859
        • Bosch F.X.
        • Ribes J.
        • Díaz M.
        • Cleries R.
        Primary liver cancer: worldwide incidence and trends.
        Gastroenterology. 2004; 127: S5-S16
        • Moller T.R.
        • Garwicz S.
        • Corazziari I.
        • Magnani C.
        Survival of children with liver tumours in Europe 1978–1989.
        Eur J Cancer. 2001; 37: 744-749
        • 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 ACCIS project): an epidemiological study.
        Lancet. 2004; 364: 2097-2105
        • Kramárová E.
        • Stiller C.A.
        The international classification of childhood cancer.
        Int J Cancer. 1996; 68: 759-765
      3. Kirkwood B.R. Sterne A.C. Essential medical statistics. Blackwell Science Ltd, Oxford2003
      4. Parkin DM, Stiller CA, Draper GJ, et al., editors. International incidence of childhood cancer (IARC scientific publications no. 87). Lyon: International Agency for Research on Cancer; 1988.

        • Pang D.
        • McNally R.
        • Birch J.M.
        • on behalf of the UK Childhood Cancer Study Investigators
        Parental smoking and childhood cancer: results from the United Kingdom Childhood Cancer Study.
        Br J Cancer. 2003; 88: 373-381
        • Sorahan T.
        • Lancashire R.J.
        Parental cigarette smoking and childhood risks of hepatoblastoma: OSCC data.
        Br J Cancer. 2004; 90: 1016-1018
        • Graafmans W.C.
        • Richardus J.H.
        • Borsboom G.J.J.M.
        • et al.
        Birth weight and perinatal mortality: a comparison of ‘optimal’ birth weight in seven Western European countries.
        Epidemiology. 2002; 13: 569-574
        • Cavelaars A.E.
        • Kunst A.E.
        • Geurts J.J.
        • et al.
        Educational differences in smoking: international comparison.
        BMJ (Clin Res Ed). 2000; 320: 1102-1107
        • Darbari A.
        • Sabin K.M.
        • Shapiro C.N.
        • Schwarz K.B.
        Epidemiology of primary hepatic malignancies in U.S. children.
        Hepatology. 2003; 38: 560-566
        • Evans A.E.
        • Land V.J.
        • Newton W.A.
        • et al.
        Combination chemotherapy (vincristine, adriamycin, cyclophosphamide, and 5-fluorouracil) in the treatment of children with malignant hepatoma.
        Cancer. 1982; 50: 821-826
        • 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
      5. Bulterys M, Goodman MT, Smith MA, Buckley JD. Hepatic tumors. In: Gloeckler Ries LA, Smith MA, Gurney JG, Linet M, Tamra T, Young JL, et al., editors. Cancer incidence and survival among children and adolescents: United States SEER Program 1975–1995. Bethesda, MD: NIH Pub No 99-4649; 1999, p. 91–7.

        • Pritchard J.
        • Brown J.
        • Shafford E.
        • et al.
        Cisplatin, doxorubicin, and delayed surgery for childhood hepatoblastoma: a successful approach —- results of the first prospective study of the International Society of Pediatric Oncology.
        J Clin Oncol. 2000; 18: 3819-3828
        • Perilongo G.
        • Shafford E.
        • Maibach R.
        • et al.
        Risk-adapted treatment for childhood hepatoblastoma: final report of the second study of the International Society of Paediatric Oncology – SIOPEL 2.
        Eur J Cancer. 2004; 40: 411-421
        • von Schweinitz D.
        • Byrd D.J.
        • Hecker H.
        • et al.
        Efficiency and toxicity of ifosfamide, cisplatin and doxorubicin in the treatment of childhood hepatoblastoma.
        Eur J Cancer. 1997; 33: 1243-1249
        • Fuchs J.
        • Rydzynski J.
        • von Schweinitz D.
        • et al.
        Pretreatment prognostic factors and treatment results in children with hepatoblastoma: a report from the German Cooperative Pediatric Liver Tumor Study HB 94.
        Cancer. 2002; 95: 172-182
        • Czauderna P.
        • Mackinlay G.
        • Perilongo G.
        • et al.
        Hepatocellular carcinoma in children: Results of the first prospective study of the International Society of Pediatric Oncology Group.
        J Clin Oncol. 2002; 20: 2798-2804
        • Farhi D.C.
        • Shikes R.H.
        • Murari P.J.
        • Silverberg S.G.
        Hepatocellular carcinoma in young people.
        Cancer. 1983; 52: 1516-1525