| Summary: | Nikolaos Machairiotis,1 Ioanna Kougioumtzi,1 Paul Zarogoulidis,2 Aikaterini Stylianaki,1 Konstantinos Tsimogiannis,3 Nikolaos Katsikogiannis11Surgery Department (National Health System), University General Hospital of Alexandroupolis, Alexandroupolis, 2Pulmonary Department – Oncology Unit, “G Papanikolaou” General Hospital, Aristotle University of Thessaloniki, Thessaloniki, 3Department of Surgery, G Hatzikosta General Hospital of Ioannina, Ioannina, GreeceAbstract: Gastrointestinal stromal tumors (GISTs) are relatively rare neoplasms of the gastrointestinal tract originating from the pluripotential mesenchymal stem cells, which differentiate into interstitial Cajal cells. They are usually located in the upper gastrointestinal track. These tumors are typically defined by the expression of c-KIT (CD117) and CD34 proteins in the tumor cells. A small percentage of these tumors is negative for c-KIT. The neoplasms are positive for platelet-derived growth factor α (PDGFα) mutations. In addition to PDGFRα mutations, wild-type c-KIT mutations can also be present. The therapeutic approach to locally developed gastrointestinal stromal tumors is surgical resection, either with open or laparoscopic surgery. In case of systemic disease, molecular pharmacologic agents such as imatinib and sunitinib are used for treatment. These agents block the signaling pathways of neoplastic-cell tyrosine kinases, interfering in their proliferation and causing apoptosis.Keywords: GIST, mesenchymal stem cells, cancer pathways, interstitial cells of Cajal, PDGFRα
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