Introduction: Gastrointestinal stromal tumors (GIST) are specific mesenchymal tumors of the gastrointestinal tract. Most of GISTs (95%) result from activating mutations in one of the receptor tyrosine kinase proteins (KIT). Tumor cells express this protein. GIST is most common in the stomach and small intestine, but may occur anywhere in the gastrointestinal tract and intra-abdominal soft tissues. The variety of its clinical presentations is related to localization of the tumor, its size and relationship to surrounding organs. Surgery is the first choice of treatment for patients with localized or potentially resectable tumors with the intention of R0 resection. Targeted therapy with imatinib (a selective inhibitor of the KIT protein) is the primary option for patients with metastatic GIST, as adjuvant treatment after surgery or neo-adjuvant therapy prior to surgery in indicated cases. This paper describes comprehensive therapy of GIST with an emphasis laid on the status of surgical treatment, and it highlights some controversial topics, e.g. the role of surgery for metastatic disease or neo-adjuvant targeted therapy.

Conclusion: GIST is a relatively rare tumor most commonly affecting the stomach and small intestine. Surgical treatment is not replaceable in the treatment strategy of this disease. The diagnostic-therapeutic approach to patients with GIST must be comprehensive. Due to prescribing restrictions of biological therapies, these patients are inevitably directed to specialized centers where surgical treatment should also be preferably provided.

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