title: Gastrointestinal stromal tumor: advances in diagnosis and management.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  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.
  
  
  METHODS
  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.
authors.affiliation: Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio 44195, USA.
authors.forename: Deepa T
authors.initials: DT
authors.lastname: Patil
authors.forename: Brian P
authors.initials: BP
authors.lastname: Rubin
mesh_terms: D001549:Benzamides; D003937:Diagnosis, Differential; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis; D046152:Gastrointestinal Stromal Tumors / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D000068877:Imatinib Mesylate; D007211:Indoles / Q000627:therapeutic use; D009154:Mutation; D010879:Piperazines / Q000627:therapeutic use; D010982:Platelet-Derived Growth Factor / Q000235:genetics / Q000378:metabolism; D011379:Prognosis; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics / Q000378:metabolism; D011743:Pyrimidines / Q000627:therapeutic use; D011758:Pyrroles / Q000627:therapeutic use; D000077210:Sunitinib
chemical_list: D001549:Benzamides; D007211:Indoles; D010879:Piperazines; D010982:Platelet-Derived Growth Factor; D011743:Pyrimidines; D011758:Pyrroles; C075278:platelet-derived growth factor A; D000068877:Imatinib Mesylate; D019009:Proto-Oncogene Proteins c-kit; D000077210:Sunitinib