title: The GIST paradigm: how to establish diagnostic and prognostic criteria.
abstract: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. The present review evaluates the most important parameters in GIST: epidemiology, principal clinical presentations, histopathological patterns of GIST with differential diagnosis and Fletcher's and Miettinen's risk classification, immunohistochemistry, prognostic factors, c-KIT and PDGFRalpha mutations and treatment of this tumor. The most frequent site for GISTs is the stomach, followed by the duodenum and small intestine; spindle cell morphology is described in 70% with CD17 positivity in around 95% of the cases; exon 11 of c-KIT is the most mutated, with Gleevec being the principal treatment. In GISTs, high mitotic rate, large tumor size, incomplete resection, extragastrointestinal site, duplication 502-503 in exon 9 of c-KIT, and secondary mutations are negative prognostic factors.
authors.affiliation: Department of Pathology, University of Valencia, Valencia, Spain.
authors.forename: S
authors.initials: S
authors.lastname: Calabuig-Farinas
authors.forename: J A
authors.initials: JA
authors.lastname: Lopez-Guerrero
authors.forename: A
authors.initials: A
authors.lastname: Llombart-Bosch
mesh_terms: D003937:Diagnosis, Differential; D005770:Gastrointestinal Neoplasms / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology / Q000601:surgery; D046152:Gastrointestinal Stromal Tumors / Q000235:genetics / Q000378:metabolism / Q000401:mortality / Q000473:pathology / Q000601:surgery; D006801:Humans; D009154:Mutation; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics / Q000378:metabolism; D015996:Survival Rate
chemical_list: D019009:Proto-Oncogene Proteins c-kit