Gastrointestinal Stromal Tumors of the Small Intestine

Abstract

Gastrointestinal stromal tumor (GIST) is the most common type of sarcoma and the third most common malignant small bowel tumor. After the stomach, the small bowel is the second most common location for GISTs. Although a majority of GISTs are driven by KIT oncogene mutations, there is increasing evidence that GIST is a genetically heterogeneous disease. Noninvasive imaging provides useful anatomic information but is not adequate to diagnose GIST. Endoscopic ultrasonography–guided tissue biopsy is the preferred method of diagnosis. GISTs are primarily treated with surgical resection, whereas adjuvant tyrosine kinase inhibitor therapy with imatinib is reserved for high-risk cases or in the setting of metastatic disease. Minimally invasive surgical approaches are safe and do not compromise oncologic outcomes in experienced centers. Selective cytoreductive surgery for metastatic GIST remains controversial but may have a role in appropriately selected patients. This review contains 10 figures, 9 tables and 65 references Key words: cytoreductive surgery, gastrointestinal stromal tumor, imatinib, interstitial cell of Cajal, KIT oncogene, minimally invasive surgery, molecular profile, small bowel