Gastrointestinal stromal tumors (GISTs) though relatively rare are the most common mesenchymal tumors of the gastrointestinal tract. In the past treatment was mainly confined to surgical resection. These tumors are relatively resistant to chemotherapy and radiotherapy. Recent understanding of pathogenesis especially the role of gain of function KIT gene mutations has enabled the rationale development of effective targeted therapies such imatinib mesylate in a relatively short period of time for both the advanced and adjuvant settings. Sunitinib, a multikinase inhibitor, has also been proven as second line therapy and other biological agents such as nilotinib and sorafenib are in clinical testing. Mutational analysis of GIST contributes predictive and prognostic information helping to guide biological therapies. The multidisciplinary management of GIST has also challenged some traditional oncological paradigms.
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