Childhood central nervous system tumours – incidence and survival in Europe (1978–1997): Report from Automated Childhood Cancer Information System project


      This paper describes the incidence and survival of childhood central nervous system (CNS) tumours in Europe for the period 1978–1997. A total of 19,531 cases, aged 0–14 years, from the ACCIS database were analysed by five regions: the British Isles, East, North, South, and West. Overall age-standardised incidence rate (ASR) of CNS tumours in Europe (1988–1997) was 29.9 per million, with the highest rates in the North. Astrocytoma (ASR = 11.8), primitive neuroectodermal tumours (PNET) (ASR = 6.5) and ependymoma (ASR = 3.4) were the most frequent types. Incidence increased significantly during 1978–1997, on average by 1.7% per year. Diagnostic methods may partially explain incidence rates and trends, although a role of variations in risk factors cannot be excluded. Overall 5-year survival was 64% and varied between 72% in the North and 53% in the East. PNET had the poorest prognosis (49%) and astrocytoma the best (75%). Survival has improved by 29% since late 1970s. The positive trends were seen in all regions, although the interregional differences persisted, as a reflection of the different healthcare systems.


      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. International Incidence of childhood cancer. vol. II. International Agency for Research on Cancer, Lyon1998
        • Stiller C.A.
        • Nectoux J.
        International incidence of childhood brain and spinal tumours.
        Int J Epidemiol. 1994; 23: 458-464
        • McKinney P.A.
        • Feltbower R.G.
        • Parslow R.C.
        • Lewis I.J.
        • Glaser A.W.
        • Kinsey S.E.
        Patterns of childhood cancer by ethnic group in Bradford, UK 1974–199.
        Eur J Cancer. 2003; 39: 92-97
        • Reutfors J.
        • Kramárová E.
        • Weiderpass E.
        • et al.
        Central nervous system tumours in children in Costa Rica, 1981–96.
        Paediatr Perinat Epidemiol. 2002; 16: 219-225
        • Martos M.C.
        • Olsen J.H.
        Childhood cancer mortality in the European Community, 1950–1989.
        Eur J Cancer. 1993; 29A: 1783-1789
        • Ries L.A.G.
        • Eisner M.P.
        • Kosary C.L.
        • et al.
        SEER cancer statistics review, 1973–1998.
        MD, National Cancer Institute, Bethesda2001
        • Magnani C.
        • Aareleid T.
        • Viscomi S.
        • Pastore G.
        • Berrino F.
        • the EUROCARE Working Group
        Variations in survival of children with central nervous system (CNS) malignancies diagnosed in Europe between 1978 and 1992: the EUROCARE study.
        Eur J Cancer. 2001; 37: 711-721
        • Ajiki W.
        • Tsukuma H.
        • Oshima A.
        Survival rates of childhood cancer patients in Osaka, Japan.
        Jpn J Clin Oncol. 2004; 34: 50-54
        • Anderson D.M.
        • Rennie K.M.
        • Neglia J.P.
        • Robison L.R.
        • Gurney J.G.
        Medical and neurocognitive late effects among survivors of childhood central nervous system tumors.
        Cancer. 2001; 92: 2709-2719
        • Scott J.N.
        • Rewcastle N.B.
        • Brasher P.M.
        • et al.
        Long-term glioblastoma multiforme survivors: a population-based study.
        Can J Neurol Sci. 1998; 25: 197-201
        • Strother D.
        • Pollack I.F.
        • Fisher P.G.
        • et al.
        Tumors of the central nervous system.
        in: Pizzo P.A. Poplack D.G. Principles and practice of pediatric oncology. Lippincott, Philadelphia2002: 751-824
        • 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 1970s: the ACCIS project.
        Lancet. 2004; 364: 2097-2105
      2. Kramárová E, Stiller CA, Ferlay J, et al. International classification of childhood cancer 1996. IARC technical report no. 29. Lyon: International Agency for Research of Cancer; 1996.

        • Giles F.H.
        • Sobel E.L.
        • Leviton A.
        • Tavaré C.J.
        • Hedley-Whyte E.T.
        Childhood Brain Tumor Consortium. Histologic feature reliability in childhood neural tumours.
        J Neuropathol Exp Neurol. 1994; 53: 559-571
        • WHO
        International classification of diseases for oncology (ICD-O).
        World Health Organisation, Geneva1976
      3. Fritz A. Percy C. Jack A. International classification of diseases for oncology. WHO, Geneva2000
      4. Tyczynski JE, Demarèt E, Parkin DM. Standards and guidelines for cancer registration in Europe. The ENCR recommendations. Vol. I. Lyon: IARC Technical Publications no. 40; 2003.

        • Gurney J.G.
        • Wall D.A.
        • Jukich P.J.
        • Davis F.G.
        The contribution of nonmalignant tumors to CNS tumor incidence rates among children in the United States.
        Cancer Causes and Control. 1999; 10: 101-105
      5. Surveillance, Epidemiology, and End Results (SEER) Program ( SEERStat Database: Incidence – SEER 9 Regs Public-Use, Nov 2003 Sub (1973–2001). Bethesda, MD: National Cancer Institute, DCCPS, Surveillance Research Program, Cancer Statistics Branch; released April 2004, based on the November 2003 submission.

        • American Cancer Society
        Manual of tumor nomenclature and coding (MOTNAC).
        American Cancer Society, New York1951
        • Smith M.A.
        • Freidlin B.
        • Ries L.A.G.
        • Simon R.
        Trends in reported incidence of primary malignant brain tumors in children in the United States.
        J Natl Can Inst. 1998; 90: 1269-1277
        • Kallio M.
        The incidence, survival, and prognostic factors of patients with intracranial glioma and meningioma in Finland from 1953 to 1987.
        Department of Neurology, University of Helsinki, Helsinki1993
        • Dreifald A.C.
        • Carlberg M.
        • Hardell L.
        Increasing incidence rates of childhood malignant diseases in Sweden during the period 1960–1998.
        Eur J Cancer. 2004; 40: 1351-1360
        • Kaatsch P.
        • Rickert C.H.
        • Külm J.
        • Schüz J.
        • Michaelis J.
        Population-based epidemiologic data on brain tumors in German children.
        Cancer. 2001; 92: 3155-3164
      6. Gurney JG, Smith MA, Bunin GR. CNS and miscellaneous intracranial and intraspinal neoplasms. In: Ries LAG, Smith MA, Gurney JG, et al., editors. Cancer incidence and survival among children and adolescents: United States SEER program 1975–1995. Bethesda, MD: National Cancer Institute, SEER Program, NIH Publication No. 99–4649; 1999, p. 51–63.

        • Hjalmars U.
        • Kulldorf M.
        • Wahqvist Y.
        • Lannering B.
        Increased incidence rates but no space-time clustering of childhood astrocitoma in Sweden, 1973–92.
        Cancer. 1999; 85: 2077-2090
        • McNally R.J.Q.
        • Kelsey A.M.
        • Cairns D.P.
        • Taylor G.M.
        • Eden O.B.
        • Birch J.M.
        Temporal increases in the incidence of childhood solid tumours seen in Northwest England (1954–1998) are likely to be real.
        Cancer. 2001; 92: 1967-1976
        • Linet M.S.
        • Ries L.A.G.
        • Smith M.A.
        • Tarone R.E.
        • Devesa S.S.
        Cancer surveillance series: recent trends in childhood cancer incidence and mortality in the United States.
        J Natl Can Inst. 1999; 91: 1051-1058
        • Kramárova E.
        • Plesko I.
        • Black R.J.
        • Obsttniková A.
        Improving survival for childhood cancer in Slovakia.
        Int J Cancer. 1996; 65: 594-600
        • Stiller C.A.
        • Bunch K.J.
        Brain and spinal tumours in children aged under two years: incidence and survival in Britain, 1971–85.
        Br J Cancer. 1992; 18: 50-53
        • Pannelli F.
        • Mosciatti P.
        • Felici L.
        • Magnani C.
        • Pacucci C.
        • Pastore G.
        Survival trends of childhood cancer during the period 1978–1994 in Italy: a first report from the Italian cancer registries.
        Epidemiol Prev. 2001; 25: 354-358
        • Gatta G.
        • Capocaccia R.
        • Stiller C.
        • et al.
        Childhood cancer survival trends in Europe: An EUROCARE Working Group Study.
        J Clin Oncol. 2005; 23: 3742-3751
        • Stiller C.A.
        Population-based survival rates for childhood cancer in Britain, 1980–91.
        BMJ. 1994; 309: 1612-1616
        • Stiller C.A.
        Centralisation of treatment and survival rates for cancer.
        Arch Dis Child. 1988; 63: 3-30
        • Selby P.
        • Gillis C.
        • Haward R.
        Benefits from specialised cancer care.
        Lancet. 1996; 348: 313-318
        • Terracini B.
        • Coebergh J.W.
        • Gatta G.
        • et al.
        Childhood cancer survival in Europe: an overview.
        Eur J Cancer. 2001; 37: 810-816
        • Gatta G.
        • Corazziari I.
        • Magnani C.
        • et al.
        Childhood cancer survival in Europe.
        Ann Oncol. 2003; 14: v119-v127
        • Mckinney P.A.
        • Feltbower R.G.
        • Parslow R.C.
        • et al.
        Survival from childhood cancer in Yorkshire, UK: effect of ethnicity and socioeconomic status.
        Eur J Cancer. 1999; 13: 1816-1823
        • Kühl J.
        • Doz F.
        • Taylor R.E.
        Embryonic tumours.
        in: Walker D.A. Perilongo G. Punt J.A.G. Brain and spinal tumours of childhood. Arnold, London2004: 314-330
        • Kulkarni A.V.
        • Bouffet E.
        • Drake J.M.
        Ependymal tumours.
        in: Walker D.A. Perilongo G. Punt J.A.G. Brain and spinal tumours of childhood. Arnold, London2004: 331-344
        • Navajas A.
        • Fernandez-Teijeiro A.
        Embryonic tumours of the central nervous system.
        Clin Transl Oncol. 2005; 7: 219-227