Genetics Of Neuroepithelial Brain Tumours

  • C. C. Tijssen
Part of the Developments in Oncology book series (DION, volume 52)


Various etiologies, such as chemical carcinogens and oncogenic viruses, have been postulated to be important in the development of neuroepithelial brain tumours. However, their exact pathogenesis remains unknown and the influence of genetic factors is uncertain. A possible relationship between genetic factors and cerebral tumour development has been under discussion since as early as 1896 when Besold reported two sisters suffering from brain tumours (1). Koch in 1954 thought two factors at least necessary for the formation of a “glioma”: 1. a local hereditary influence: neuroglial dysplasia in the region of the ventricular germinal centres with a tendency to tumour degeneration; he suggested a mutation in a pleiotropic gene to be responsible for this local disturbance. 2. a second or several factors, exogenous or endogenous, that initiate oncogenesis in these predisposed areas (2). This corresponds with the two-hit hypothesis later put forward by Knudson which supposes that cancer results from at least two events one of which could be transmitted genetically (3). In the hereditary forms of cancer, the first mutation occurs in a germinal cell and somatic mutations later occur at the same site, converting proto-oncogenes to oncogenes.

Key words

Brain Tumours Neuroepithelial tissue Genetics Familial occurrence 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Besold (G.): Ueber zwei Fälle von Gehirntumor bei zwei Geschwistern. Dtsch Z Nervenheilk 8: 49–74 (1986).CrossRefGoogle Scholar
  2. 2.
    Koch (G.): Beitrag zur Erblichkeit der Hirngeschwülste. Acta Genet Med Gemellol 3: 170–191 (1954).Google Scholar
  3. 3.
    Knudson (A.G.), Strong (L.C.), Anderson (D.E.): Heredity and cancer in man. Prog Med Genet 9: 113–158 (1973).PubMedGoogle Scholar
  4. 4.
    Tijssen (C.C.), Halprin (M.R.), Endtz (L.J.): Familial Brain Tumours (Martinus Nijhoff, 1982).CrossRefGoogle Scholar
  5. 5.
    International Register of Familial Brain Tumours (IRFBT): Comprehensive Cancer Centre, Opaalstraat 5, 2332 TA Leyden, The Netherlands.Google Scholar
  6. 6.
    Tupchong (L.), Levison (D.A.), Jones (A.E.): Concomitant Conjugal Gliomas with Similar Histologic Features. Cancer 55: 864–869 (1985).PubMedCrossRefGoogle Scholar
  7. 7.
    Griffin (T.W.), Smith (T.W.), Levy (B.S.), Recht (L.D.): Synchronous occurrence of glioblastoma multiforme in a husband and wife. J. Neuro-Onc 4: 75–78 (1986).CrossRefGoogle Scholar
  8. 8.
    Draper (G.J.), Heaf (M.M.), Kinnier Wilson (L.M.): Occurrence of childhood cancers among sibs and estimation of familial risks. J. Med Gen 14: 81–90 (1977).CrossRefGoogle Scholar
  9. 9.
    Farwell (J.), Flannery (J.T.): Cancer in relatives of children with central-nervous-system neoplasms. N. Engl J Med 311: 749–753 (1984).PubMedCrossRefGoogle Scholar
  10. 10.
    Painter (M.J.), Pang (D.), Ahdab-Barmada (M.), Bergman (J.): Connatal brain tumors in patients with tuberous sclerosis. Neurosurgery 14: 570–573 (1984).PubMedCrossRefGoogle Scholar
  11. 11.
    Bigner (S.H.), Bjverkvig (R.), Laerum (O.D.): DNA content and chromosal composition of malignant human gliomas. Neuro Clin 3: 769–784 (1985).Google Scholar
  12. 12.
    Shapiro (J.R.): Biology of gliomas: heterogeneity, oncogenes, growth factors. Semin Oncol 13: 4–15 (1986).PubMedGoogle Scholar
  13. 13.
    Chadduck (W.N.), Netsky (M.G.): Familial gliomas: report of four families, with chromosome studies. Neurosurgery 10: 445–449 (1982).PubMedCrossRefGoogle Scholar
  14. 14.
    Challa (V.R.), Goodman (H.O.), Davis (C.H.): Familial Brain Tumors: Studies of Two Families and Review of Recent Literature. Neurosurgery 12: 18–23 (1983).PubMedCrossRefGoogle Scholar
  15. 15.
    Leblanc (R.), Lozano (A.), Robitaille (Y.): Familial mixed oligodendrocytic-astrocytic gliomas. Neurosurgery 18: 480–482 (1986).PubMedCrossRefGoogle Scholar

Copyright information

© Martinus Nijhoff Publishers, Dordrecht 1987

Authors and Affiliations

  • C. C. Tijssen
    • 1
  1. 1.Department of NeurologySt. Elisabeth and Maria HospitalsTilburgThe Netherlands

Personalised recommendations