Gastrointestinal Stromal Tumors: A Review of Case Reports, Diagnosis, Treatment, and Future Directions
Gastrointestinal stromal tumors (GISTs), the most common sarcoma of the GI tract, have unique kinase mutations that serve as targets for medical therapy. This article reviews the data supporting the use of the tyrosine kinase inhibitor (TKI) imatinib in GIST patients, and how this treatment should be combined with surgical resection (when possible) to optimize patient outcomes. Although surgical resection remains the mainstay of treatment for these tumors, patients with resected GISTs have high relapse rates that can be reduced by 1 year of adjuvant imatinib. Data also support the use of imatinib for patients with recurrent or unresectable GIST. In these patients the drug should be continued until progression, intolerance, or the patients are rendered resectable. Patients with advanced GIST who are successfully resected after imatinib treatment should be placed back on imatinib postoperatively. Patients who develop generalized progression (progression at 2 or more sites) on imatinib should move to other treatments, such as newer TKIs or other targeted approaches currently under study. Genotyping of the tumor should be considered in all pediatric GISTs and high risk adult GISTs, especially if there is progression on imatinib. Quality of life and the cost/benefit of new therapies are important issues for further study in patients with GIST.