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Chapter-076 Familial Tumor Syndromes of the Central Nervous System

BOOK TITLE: Textbook of Contemporary Neurosurgery (2 Volumes)

Author
1. Mittal Sandeep
2. Mittal Monika
3. Delly Fadi
4. Beaumont Thomas L
ISBN
9789350252390
DOI
10.5005/jp/books/11681_76
Edition
1/e
Publishing Year
2012
Pages
39
Author Affiliations
1. Wayne State University, Detroit, MI, USA
2. Wayne State University, Detroit, MI, USA
3. Wayne State University, Detroit, MI, USA
4. Wayne State University, Detroit, MI, USA
Chapter keywords

Abstract

Several familial syndromes are associated with an increased incidence of neoplasms of the nervous system. Timely and accurate identification of these syndromes is essential to provide adequate medical care and counseling to affected individuals and their families. There has been tremendous progress in our understanding of the genetic alterations that underlie these relatively uncommon disorders. Our grasp of the molecular pathways involved in tumorigenesis has contributed to the development of various targeted therapies. In this chapter, review the common hereditary syndromes associated with brain tumors including neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), tuberous sclerosis complex (TSC) and Von Hippel-Lindau (VHL) disease, as well as, the less frequently seen Gorlin syndrome, Li-Fraumeni syndrome, Cowden syndrome and Turcot syndrome.

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