Document Detail


Hereditary neuroendocrine tumors of the gastroenteropancreatic system.
MedLine Citation:
PMID:  17684762     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Approximately 5-10% of neuroendocrine tumors (NETs) of the gastroenteropancreatic system (GEP) have a hereditary background. The known inherited syndromes include multiple endocrine neoplasia type 1, neurofibromatosis type 1, von Hippel-Lindau disease, and the tuberous sclerosis complex. This review discusses for each of these syndromes the: (1) involved genes and specific types of mutations, (2) disease prevalence and penetrance, (3) affected neuroendocrine tissues and related clinical syndromes, (4) special morphological features of NETs and their putative precursor lesions. In addition, GEP-NETs clustering in individual families or associated with other malignancies without known genetic background are discussed.
Authors:
Martin Anlauf; Nele Garbrecht; Juliane Bauersfeld; Anja Schmitt; Tobias Henopp; Paul Komminoth; Philipp U Heitz; Aurel Perren; Günter Klöppel
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review     Date:  2007-08-08
Journal Detail:
Title:  Virchows Archiv : an international journal of pathology     Volume:  451 Suppl 1     ISSN:  0945-6317     ISO Abbreviation:  Virchows Arch.     Publication Date:  2007 Aug 
Date Detail:
Created Date:  2007-08-20     Completed Date:  2007-12-27     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9423843     Medline TA:  Virchows Arch     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  S29-38     Citation Subset:  IM    
Affiliation:
Department of Pathology, University of Kiel, Michaelisstr. 11, 24105, Kiel, Germany. manlauf@path.uni-kiel.de
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MeSH Terms
Descriptor/Qualifier:
Digestive System Neoplasms / genetics*,  pathology
Genetic Predisposition to Disease*
Humans
Neuroendocrine Tumors / genetics*,  pathology

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