![]() ![]() With the discovery of mutations associated with these tumors, the treatment has changed dramatically. Traditional chemotherapy and radiation are not effective on GISTs, therefore surgical resection has always been the mainstay of treatment ( 5, 6). Although GISTs are considered rare tumors, most GISTs are discovered incidentally so the true prevalence is unknown. Hirota and colleagues discovered that these tumors express CD117 antigen (C-Kit), a gain of function mutation responsible for activating the growth of these tumors ( 4). Kindblom and associates in 1998 found that these tumors actually originate from the interstitial cells of Cajal ( 3). Gastrointestinal stromal tumors (GISTs) were originally believed to have originated from the mesenchymal cells of the gastrointestinal tract (GIT) ( 1, 2). This report addresses the epidemiology, clinical presentation, diagnostic imaging, histologic diagnosis, classification and risk stratification, staging and grading, surgical treatment, adjuvant treatment, and metastasis of GISTs. Newer therapies of ipilimumab, nivolumab, and endoscopic ultrasound alcohol ablation have shown promising results. ![]() Radiofrequency ablation has shown to be effective when surgery is not suitable. High-risk tumors should be monitored for recurrence with serial abdominal CT scans. Sunitinib is required for KIT exon 9, 13, and 14 mutations, while ponatinib is used for exon 17 mutations and regorafenib for highly refractory tumors. The approaches to treating GISTs are to resect primary low-risk tumors, resect high-risk primary or metastatic tumors with imatinib 400 mg daily for 12 months, or if the tumor is unresectable, neoadjuvant imatinib 400 mg daily followed by surgical resection is recommended. There have been many risk stratification classifications systems which are calculated based on tumor size, mitotic rate, location, and perforation. ![]() They are best identified by computed tomography (CT) scan and most stain positive for CD117 (C-Kit), CD34, and/or DOG-1. Gastrointestinal stromal tumors (GISTs) are rare neoplasms of the gastrointestinal tract associated with high rates of malignant transformation. ![]()
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