ABSTRACT
The management of gastrointestinal stromal tumors (GISTs) represents one of the most exciting and rapidly advancing fields in gastrointestinal oncology. Although GISTs were declared a distinctive clinico-pathological entity in 1983, their clinical management has been recently revolutionized by the introduction of effective molecularly targeted therapy. The tyrosine kinase KIT, recognized to be essential in the pathogenesis of GISTs, has become not only a marker for diagnosis but also a target for drug therapy. While surgical resection previously offered the only hope for patients with GISTs, their optimal management today integrates surgery with drug therapy in a multidisciplinary approach. It is important to realize that much of the evidence basis for the clinical management of GIST has not matured. As the current diagnostic criteria for GISTs were not formally defined until the National Institute of Health (NIH) consensus meeting in 2001, most retrospective studies are contaminated by non-GIST tumors (1). Prospective studies, mostly initiated after 2000, have limited follow-up or are ongoing or emerging. This chapter aims to review the available literature surrounding the molecular pathology of GISTs, their clinical characteristics, as well as the current role of surgery for patients presenting with primary disease or with metastatic/recurrent disease. The development and the clinical efficacy of targeted drug therapy are discussed in detail in the following chapter and will not constitute the main focus of this chapter.
