Genetic causes of brain tumors: neurofibromatosis, tuberous sclerosis, von Hippel-Lindau, and other syndromes.

Publication/Presentation Date

11-1-2007

Abstract

Several familial syndromes are associated with an increased incidence of nervous system tumors. Recognition of these syndromes is critical to provide optimal clinical care and genetic counseling to affected patients and their families. Identification of the genetic defects responsible for these relatively uncommon disorders has led to the improved understanding of critical molecular pathways involved in tumorigenesis and has contributed to the emergence of molecularly targeted therapeutics against cancer. The hereditary syndromes and diseases included in this review are limited to those associated with brain tumors: neurofibromatosis 1, neurofibromatosis 2, tuberous sclerosis complex, von Hippel-Lindau disease, and the less frequently encountered Cowden disease and Li-Fraumeni, Turcot's, and Gorlin's syndromes.

Volume

25

Issue

4

First Page

925

Last Page

946

ISSN

0733-8619

Disciplines

Medicine and Health Sciences

PubMedID

17964021

Department(s)

Department of Surgery

Document Type

Article

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