Skip to main content
Log in

Familial gliomas

Analysis of six families with cerebral gliomas and without other inheritable syndromes

  • Neurooncology
  • Published:
Neurosurgical Review Aims and scope Submit manuscript


Although a familial tendency to develop brain tumors in the absence of well-recognized hereditary disorders is an important issue, it has been rarely reported. Studies of the familial occurrence of gliomas have yielded conflicting results. This phenomenon may be imputable to chance or environmental or hereditary factors. Furthermore, epidemiological and genetic investigations are fundamental to evidence of whether hereditary factors are active in the development of these tumors. We describe a group of six families, in each of which more than one member had glioma, and discuss the probable mechanisms of oncogenesis in familial tumors in light of the pertinent literature.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others


  1. Eng C, Ponder BR (1993) The role of gene mutations in the genesis of familial cancer. FASEB J 7:910–919

    CAS  PubMed  Google Scholar 

  2. Knudson AG (1993) Antioncogenes and human cancer. Proc Natl Acad Sci U S A 90:10914–10921

    CAS  PubMed  Google Scholar 

  3. Kelley MJ, Jonhson BE (1994) Genetic mechanism of solid tumorigenesis. Adv Intern Med 39:93–122

    CAS  PubMed  Google Scholar 

  4. Tomatis L (1988) Environmental cancer risk factors: a review. Oncology 27:465–472

    CAS  Google Scholar 

  5. Jones SM, Phillips PC, Molloy PT, Lange BJ, Needle MN, Bunin GR, Biegel JA (1995) Neurologic and other disorders in relatives of pediatric patients with CNS tumors. Pediatr Neurol 13:11–116

    Article  Google Scholar 

  6. Paunu N, Syrjakoski K, Sankila R, Simola KOJ, Helen P, Niemela M, Matikainen M, Isola J, Haapasolo H (2002) Analysis of p 53 tumor suppressor gene in families with multiple glioma patients. J Neurooncol 55:159–165

    Google Scholar 

  7. Central Brain Tumor Registry of the United States (1997) 1996 annual report

  8. Ikizer Y, van Meyel DJ, Ramsay GL, Ramsay DA, Abdallah GL, Allaster RM, MacDonald DR, Cavenee WK, Cairncross JG (1992) Gliomas in families. Can J Neurol Sci 19:492–497

    PubMed  Google Scholar 

  9. Tops CM, Vasen HF, van-Berge-Henegouwen G (1992) Genetic evidence that Turcot syndrome is not allelic to familial adenomatous polyposis. Am J Med Genet 43:888–893

    CAS  PubMed  Google Scholar 

  10. Rodriguez HA, Berthrong M (1966) Multiple primary intracranial tumor in Von Recklinghausen's neurofibromatosis. Arch Neurol 14:467–475

    CAS  PubMed  Google Scholar 

  11. Kapp JP, Paulson G, Odom GL (1967) Brain tumors with tuberous sclerosis. J Neurosurg 26:191–202

    CAS  PubMed  Google Scholar 

  12. Li FP, Fraumeni JF (1990) Soft-tissue sarcomas, breast cancer, and other neoplasm. A familial syndrome? Ann Intern Med 71:747–749

    Google Scholar 

  13. Turcot J, Despres JP, St Pierre F (1959) Malignant tumors of the central nervous system associated with familial polyposis of the colon: report of two cases. Dis Colon Rectum 2:465–468

    CAS  Google Scholar 

  14. Berleur M, Cordier S (1995) The role of chemical physical, or viral exposures and health factors in neurocarcinogenesis: implications for epidemiologic studies in brain tumors. Cancer Causes Control 6:40–256

    Google Scholar 

  15. Grossman SA, Osman M, Hrubam R, Piantadosi S (1999) Central nervous system cancers in first-degree relatives and spouses. Cancer Inv 17:299–308

    CAS  Google Scholar 

  16. Von Motz IP, Bots G, Endtz LJ (1977) Astrocytoma in three sisters. Neurology 27:1038–1041

    PubMed  Google Scholar 

  17. Heuch I, Blom GP (1986) Glioblastoma multiforme in three family members including one case of true multicentricity. J Neurol 233:142–144

    CAS  Google Scholar 

  18. Lossignol D, Grossman SA, Sheider VR et al (1990) Familial clustering of malignant astrocytomas. J Neurooncol 9:139–145

    CAS  PubMed  Google Scholar 

  19. Li FP (1990) Familial cancer syndromes and clusters. Curr Probl Cancer 14:77–83

    Google Scholar 

  20. Aita JM (1967) Genetic aspect of tumors of the nervous system. Recent Results Cancer Res 12:86–110

    Google Scholar 

  21. Harvald B, Hauge M (1956) On the heredity of glioblastoma. JNCI 17:289–296

    Google Scholar 

  22. Van der Weil HJ (1960) Inheritance of glioma. Elsevier, New York

  23. Metzel E (1964) Betrachtungen zur Genetik der familiären Gliome. Acta Genet Med Gemellol (Roma) 13:124–131

    Google Scholar 

  24. Choi NW, Schuman LM, Gullen WH (1970) Epidemiology of primary central nervous system neoplasms. II. Case control study. Am J Epidemiol 91:467–485

    CAS  PubMed  Google Scholar 

  25. de Andrade M, Barnholtz JS, Amos CI, Adatto P Spencer C, Bondy ML (2001) Segregation analysis of cancer in families of glioma patients. Genet Epidemiol 20:258–270

    Google Scholar 

  26. Isamat F, Miranda AM, Bartumeus F, Prat J (1974) Genetic implication of familial brain tumors. J Neurosurg 41:573–575

    CAS  PubMed  Google Scholar 

  27. Kaufman HH, Brisman R (1972) Familial gliomas. Report of four cases. J Neurosurg 37:110–112

    CAS  PubMed  Google Scholar 

  28. Li YJ, Sanson M, Hoang-Xuan K, Delattre JY, Poisson M, Thomas G, Hamelin R (1995) Incidence of germ-line p 53 mutations in patients with gliomas. Int J Cancer 64:383–387

    PubMed  Google Scholar 

  29. Malmer B, Gronberg H, Andersson U, Jonsson BA, Henriksson R (2001) Microsatellite instability, PNETN and p 53 germline mutations in glioma families. Acta Oncol 40:633–637

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations


Corresponding author

Correspondence to Maurizio Salvati.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Caroli, E., Salvati, M., Peruzzi, P. et al. Familial gliomas. Neurosurg Rev 26, 280–282 (2003).

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: