The discovery of the KIT and platelet derived growth factor receptor alpha (PDGFRA) tyrosine-kinase gain of function mutations, which result in continuous activation and signaling, dramatically altered the understanding and treatment of GISTs (3,4). Approximately 90% of GISTs are driven by gain-of-function mutations in one of the two type-III tyrosine kinase receptors. Although resection remains a principle therapy, tyrosine-kinase inhibitors have revolutionized the treatment of advanced and recurrent GISTs, allowing many patients with advanced disease to live many years and in some instances, obtain complete remission (5-8). Wild-type gastrointestinal stromal tumors (WT-GISTs) are defined as GISTs that lack the gain-of-function mutations in KIT and PDGFRA (9). Due to the different molecular drivers and the distinct tumor biology and behavior of these wild-type neoplasms, a different approach to treatment may be required when addressing these distinct GISTs. Epidemiology of WT-GIST Regardless of subtype, the incidence of all GISTs is approximately 14-20 per million population (1). The incidence of WT-GIST is as much as 10% of all GISTs (10). Patients with WT-GISTs usually present at a much younger
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