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Pathology Research International
Volume 2011 (2011), Article ID 708596, 7 pages
Review Article

Gastrointestinal Stromal Tumors: Molecular Mechanisms and Targeted Therapies

Departments of Anatomic and Molecular Pathology, Cleveland Clinic, 9500 Euclid Avenue, L25, Cleveland, OH 44195, USA

Received 22 December 2010; Accepted 25 January 2011

Academic Editor: Hanlin L. Wang

Copyright © 2011 Erinn Downs-Kelly and Brian P. Rubin. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.