Gastrointestinal Stromal Tumor: Advances in Diagnosis and Management

Abstract

Context.—Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract and should be differentiated from other mesenchymal tumors. They harbor specific activating mutations in the KIT or platelet-derived growth factor receptor α (PDGFRA) receptor tyrosine kinases, which makes them responsive to pharmacologic inhibitors, such as imatinib mesylate and sunitinib malate. Objectives.—To provide a comprehensive review of the pathogenesis of GIST and the underlying principles of targeted therapy, to review the salient histologic and immunohistochemical features that facilitate the distinction of GIST from other mesenchymal neoplasms of the gastrointestinal tract, and to present the prognostic parameters for risk stratification that guide clinical management. Data Sources.—Review of the English literature through PubMed as well as personal experience. Photographs were taken from cases encountered at the Cleveland Clinic. Conclusions.—The discovery of the KIT-GIST connection has not only improved the diagnostic accuracy of GISTs but also provided us with a better understanding of the histogenesis and molecular pathogenesis of these neoplasms.

Gastroduodenal Intussusception Due to Pedunculated Polypoid Gastrointestinal Stromal Tumour ( GIST ): A Rare Case

“…GIST is a non-epithelial, mesenchymal tumour first described by Mazur and Clark in 1983. Gastrointestinal stromal tumours (GISTs) account for less than 3% of all gastrointestinal tract tumours and 5.7% of all sarcomas, and the majority of these tumours are gastric in origin [1,2].…”

“…So the tumour size, location, and mitotic index remain the main variables used in risk stratification systems, which was first developed by the National Institute of health (NIH), the so-called Fletcher's criteria [2,8].…”

“…A preoperative diagnosis can be difficult and diagnosis cannot be confirmed until surgery [2]. GIST can also cause symptoms secondary to mass effect including satiety, bloating, and abdominal pain [5,6].…”

Primary gastrointestinal stromal tumors: Current advances in diagnostic biomarkers, prognostic factors and management of its duodenal location

“…The second diagnostic biomarker is CD34 which is expressed in almost 80% of gastric GIST, 50% of small intestine GIST, and 95% of esophagus and rectum GIST (47)(48)(49).…”

The clinical and histopathological presentations of patients with gastrointestinal stromal tumors

“…Malign davranışlı GİST'lerde son zamanlarda c-KİT reseptör tirozin kinaz inhibitörlerinin kullanılmasıyla hastalıksız sağkalım oranları artmıştır. [4][5][6][7] Bu çalışma da; GİST tanısı konulan olgularımı-zın klinik, patolojik ve yerleşim özelliklerinin tartı-şılması amaçlanmıştır.…”