Neurooncology of familial cancer syndromes Review uri icon


MeSH Major

  • Genetic Predisposition to Disease
  • Germ-Line Mutation
  • Inheritance Patterns
  • Neoplasms, Nerve Tissue


  • The majority of tumors of the nervous system are sporadic. However, a subset of patients with tumors and their families are predisposed to developing cancers of the central nervous system and other organs because of a germline mutation. In the last decade, many of the genes responsible for these typically autosomal dominant familial tumor syndromes have been identified. Additionally, our understanding of the mechanisms of carcinogenesis in these syndromes has increased, allowing for more targeted therapies for these patients as well as those with sporadic cancers. Because these patients present a unique set of issues regarding diagnosis and neurooncological management, the most common familial cancer syndromes involving the nervous system are reviewed: neurofibromatosis type 1 and 2; tuberous sclerosis complex; von Hippel Lindau, Li-Fraumeni, Gorlin, and Turcot syndrome.

publication date

  • December 2009



  • Review



  • eng

Digital Object Identifier (DOI)

  • 10.1177/0883073809337539

PubMed ID

  • 19955345

Additional Document Info

start page

  • 1526

end page

  • 35


  • 24


  • 12