Hereditary tumor syndromes and gliomas.

Hereditary tumor syndromes and gliomas.

MedLine Citation:	PMID: 19322539 Owner: NLM Status: MEDLINE
Abstract/OtherAbstract:	Several congenital syndromes caused by germline mutations in tumor suppressor genes predispose to the development of glial tumors. In the last few decades our knowledge about the molecular functions of these genes and the pathogenesis of hereditary tumor syndromes has greatly increased. The most common syndromes are the neurofibromatoses (type 1 and type 2) and the tuberous scleroses complex. There are interesting overlaps in the molecular pathogen-esis. Deregulation of Ras or downstream Ras pathways including MEK/ERK and AKT/ mTOR plays an important role in these three syndromes. Other rare syndromes include Li-Fraumeni, melanoma-astrocytoma, and Turcot syndrome involving cell cycle regulators and DNA repair genes. The genes and pathways involved in the pathogenesis of these syndromes also play an important role in the development of sporadic tumors. Therefore research on hereditary syndromes contributes substantially to our understanding of tumor formation.

Authors:	David Reuss; Andreas von Deimling
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Publication Detail:	Type: Journal Article; Review
Journal Detail:	Title: Recent results in cancer research. Fortschritte der Krebsforschung. Progrès dans les recherches sur le cancer Volume: 171 ISSN: 0080-0015 ISO Abbreviation: Recent Results Cancer Res. Publication Date: 2009
Date Detail:	Created Date: 2009-03-26 Completed Date: 2009-06-18 Revised Date: -
Medline Journal Info:	Nlm Unique ID: 0044671 Medline TA: Recent Results Cancer Res Country: Germany
Other Details:	Languages: eng Pagination: 83-102 Citation Subset: IM
Affiliation:	Department of Neuropathology, Institute of Pathology, Im Neuenheimer Feld 220/221, Heidelberg 69120, Germany. David.Reuss@med.uni-heidelberg.de
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MeSH Terms
Descriptor/Qualifier:	Brain Neoplasms / genetics* Glioma / genetics* Humans Li-Fraumeni Syndrome / genetics Melanoma / genetics Neoplastic Syndromes, Hereditary / genetics* Neurofibromatoses / genetics Tuberous Sclerosis / genetics

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