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. 2011 Apr 14:2011:708596.
doi: 10.4061/2011/708596.

Gastrointestinal stromal tumors: molecular mechanisms and targeted therapies

Erinn Downs-Kelly  1 , Brian P Rubin
Affiliations

Gastrointestinal stromal tumors: molecular mechanisms and targeted therapies

Erinn Downs-Kelly et al. Patholog Res Int. .

Abstract

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract and are diverse not only in their clinical behavior but also in their histologic appearance. GISTs are insensitive to conventional sarcoma chemotherapy and radiation. However GISTs are sensitive to small-molecule tyrosine kinase inhibitors as 85-90% of GISTs have KIT or platelet-derived growth factor receptor alpha (PDGFRA) mutations, which drive tumorigenesis. This review will briefly touch on the clinicopathological features of GIST, while the majority of the review will focus on the clinical and treatment ramifications of KIT and PDGFRA mutations found in GIST.

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Figures

Figure 1
Figure 1
KIT and PDGFRA with locations and frequency of activating mutations in GISTs. Exons denoted with an *represent those most frequently involved by secondary mutations. Modified with permission from Heinrich et al. [3]. Copyright 2003 by the American Society of Clinical Oncology. All rights reserved.
See this image and copyright information in PMC

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