title: Inherited predisposition to gastrointestinal stromal tumor.
abstract: Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract and are a relatively recently identified category of mesenchymal tumors. Germline mutations in a number of different genes predispose to GIST. This article discusses familial GIST syndrome, Carney triad, Carney-Stratakis syndrome, and neurofibromatosis type 1, and addresses the recognition of an inherited predisposition in GIST patients.
authors.affiliation: Department of Medicine, Clinical Genetics, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA.
authors.forename: Rinki
authors.initials: R
authors.lastname: Agarwal
authors.forename: Mark
authors.initials: M
authors.lastname: Robson
mesh_terms: D005192:Family Health; D046152:Gastrointestinal Stromal Tumors / Q000235:genetics* / Q000473:pathology; D020022:Genetic Predisposition to Disease; D006801:Humans; D009154:Mutation; D009456:Neurofibromatosis 1; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics*; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics*
chemical_list: D019009:Proto-Oncogene Proteins c-kit; D020796:Receptor, Platelet-Derived Growth Factor alpha