title: [Gastrointestinal stromal tumors (GISTs): clinical and pathological features].
abstract: Gastrointestinal stromal tumors (GISTs): clinical and pathological features. The gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. With immunohistochemical, electron microscope and molecular examinations they can be clearly distinguished in both their genotype and phenotype from other mesenchymal tumors. GIST tumors express the CD 117 receptor in more than 90% independent of histopathological features and clinical behaviour. This is why it is considered as the most important characteristic. The incidence is 10-20 new cases per 1 million annually. The number of incidents is expected to increase by the establishment of CD117 and other new markers (protein kinase C theta, DOG1). Nowadays the establishment of the expected biological behavior and malignancy can be difficult. The best prognostic factors are the tumor size and the mitotic index. Dominantly, due to the mutation of the c-kit proto-oncogene and PGDFRA gene that the high level tyrosine kinase activity generates resulting uncontrolled proliferation and cell growth. The imatinib mesylate is a selective inhibitor of the KIT tyrosine kinase receptor and it also blocks the activity of the PDGFRA kinase. The therapeutic consequence of this is that the majority of advanced GIST tumors which do not react to conventional radio- and chemotherapy respond well to tyrosine kinase inhibitor treatment. As a result, survival and patient's quality of life can significantly improve.
authors.affiliation: Vaszary Kolos Kórhaz, II. Belgyógyászati Osztály, Esztergom. drmarta.kovacs@axelero.hu
authors.forename: Márta
authors.initials: M
authors.lastname: Kovács
authors.forename: Pál
authors.initials: P
authors.lastname: Nagy
authors.forename: Gábor
authors.initials: G
authors.lastname: Pák
authors.forename: János
authors.initials: J
authors.lastname: Fehér
mesh_terms: D000075369:Anoctamin-1; D000970:Antineoplastic Agents / Q000494:pharmacology; D014408:Biomarkers, Tumor / Q000032:analysis*; D018118:Chloride Channels; D004791:Enzyme Inhibitors / Q000494:pharmacology; D046152:Gastrointestinal Stromal Tumors / Q000737:chemistry / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008565:Membrane Proteins / Q000032:analysis; D008854:Microscopy, Electron; D008940:Mitotic Index; D009154:Mutation; D009363:Neoplasm Proteins / Q000032:analysis; D011379:Prognosis; D011493:Protein Kinase C / Q000032:analysis; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors; D000090063:Proto-Oncogene Mas; D019009:Proto-Oncogene Proteins c-kit / Q000032:analysis / Q000235:genetics; D018570:Risk Assessment; D012307:Risk Factors
chemical_list: C476213:ANO1 protein, human; D000075369:Anoctamin-1; D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor; D018118:Chloride Channels; D004791:Enzyme Inhibitors; C000715470:MAS1 protein, human; D008565:Membrane Proteins; D009363:Neoplasm Proteins; D000090063:Proto-Oncogene Mas; D011505:Protein-Tyrosine Kinases; D019009:Proto-Oncogene Proteins c-kit; D011493:Protein Kinase C