title: Diagnosis of gastrointestinal stromal tumors: A consensus approach.
abstract: As a result of major recent advances in understanding the biology of gastrointestinal stromal tumors (GISTs), specifically recognition of the central role of activating KIT mutations and associated KIT protein expression in these lesions, and the development of novel and effective therapy for GISTs using the receptor tyrosine kinase inhibitor STI-571, these tumors have become the focus of considerable attention by pathologists, clinicians, and patients. Stromal/mesenchymal tumors of the gastrointestinal tract have long been a source of confusion and controversy with regard to classification, line(s) of differentiation, and prognostication. Characterization of the KIT pathway and its phenotypic implications has helped to resolve some but not all of these issues. Given the now critical role of accurate and reproducible pathologic diagnosis in ensuring appropriate treatment for patients with GIST, the National Institutes of Health convened a GIST workshop in April 2001 with the goal of developing a consensus approach to diagnosis and morphologic prognostication. Key elements of the consensus, as described herein, are the defining role of KIT immunopositivity in diagnosis and a proposed scheme for estimating metastatic risk in these lesions, based on tumor size and mitotic count, recognizing that it is probably unwise to use the definitive term "benign" for any GIST, at least at the present time.
authors.affiliation: Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston MA 02115, USA.
authors.forename: Christopher D M
authors.initials: CD
authors.lastname: Fletcher
authors.forename: Jules J
authors.initials: JJ
authors.lastname: Berman
authors.forename: Christopher
authors.initials: C
authors.lastname: Corless
authors.forename: Fred
authors.initials: F
authors.lastname: Gorstein
authors.forename: Jerzy
authors.initials: J
authors.lastname: Lasota
authors.forename: B Jack
authors.initials: BJ
authors.lastname: Longley
authors.forename: Markku
authors.initials: M
authors.lastname: Miettinen
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: O'Leary
authors.forename: Helen
authors.initials: H
authors.lastname: Remotti
authors.forename: Brian P
authors.initials: BP
authors.lastname: Rubin
authors.forename: Barry
authors.initials: B
authors.lastname: Shmookler
authors.forename: Leslie H
authors.initials: LH
authors.lastname: Sobin
authors.forename: Sharon W
authors.initials: SW
authors.lastname: Weiss
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides; D014408:Biomarkers, Tumor / Q000378:metabolism; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism; D006801:Humans; D000068877:Imatinib Mesylate; D009154:Mutation; D010879:Piperazines / Q000627:therapeutic use; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics / Q000378:metabolism; D011743:Pyrimidines / Q000627:therapeutic use; D012509:Sarcoma / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism; D017154:Stromal Cells / Q000473:pathology
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D014408:Biomarkers, Tumor; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D019009:Proto-Oncogene Proteins c-kit