Inherited predisposition to gastrointestinal stromal tumor Journal Article


Authors: Agarwal, R.; Robson, M.
Article Title: Inherited predisposition to gastrointestinal stromal tumor
Abstract: Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract and are a relatively recently identified category of mesenchymal tumors. Germline mutations in a number of different genes predispose to GIST. This article discusses familial GIST syndrome, Carney triad, Carney-Stratakis syndrome, and neurofibromatosis type 1, and addresses the recognition of an inherited predisposition in GIST patients. © 2009 Elsevier Inc. All rights reserved.
Keywords: protein expression; treatment response; gene mutation; mutation; clinical feature; histopathology; review; sunitinib; nonhuman; cancer incidence; c-kit; carney-stratakis; gastrointestinal stromal tumor; germline; neurofibromatosis; pdgfra; cd34 antigen; imatinib; platelet derived growth factor alpha receptor; stem cell factor; stem cell factor receptor; autosomal inheritance; cancer susceptibility; carney complex; carney stratakis syndrome; cell structure; demography; diagnostic procedure; disease association; drug inhibition; familial disease; gene overexpression; heredity; interstitial cell of cajal; mesenchyme cell; paraganglioma; proto oncogene; smooth muscle fiber; tumor localization; family health; gastrointestinal stromal tumors; genetic predisposition to disease; neurofibromatosis 1; proto-oncogene proteins c-kit; receptor, platelet-derived growth factor alpha
Journal Title: Hematology/Oncology Clinics of North America
Volume: 23
Issue: 1
ISSN: 0889-8588
Publisher: Elsevier Inc.  
Date Published: 2009-02-01
Start Page: 1
End Page: 13
Language: English
DOI: 10.1016/j.hoc.2008.12.003
PUBMED: 19248967
PROVIDER: scopus
DOI/URL:
Notes: --- - "Cited By (since 1996): 2" - "Export Date: 30 November 2010" - "CODEN: HCNAE" - "Source: Scopus"
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  1. Mark E Robson
    676 Robson