title: Multiple non-metastatic gastrointestinal stromal tumors. Differential features.
abstract: BACKGROUND
  gastrointestinal stromal tumors (GISTs) are specific, generally KIT (CD117)-positive, mesenchymal tumors of the digestive tract displaying KIT or PDGFRA gene mutations. Clinically, they tend to present as solitary tumors of the intestinal wall; more rarely, multiple tumors may occur in one or more organs.
  
  
  OBJECTIVE
  to review the morphological, immunohistochemical and molecular features of multiple, non-metastatic forms of GIST.
  
  
  METHODS
  review of the literature on Medline, and authors own experience.
  
  
  CONCLUSIONS
  multiples GISTs may occur in three different contexts: as spontaneous lesions (in both adults and children); due to familial GIST syndrome (autosomal dominant inheritance); or in association with specific syndromes (e.g. Carney s triad, Carney-Stratakis syndrome, type I neurofibromatosis). Outside these contexts, the existence of multiple GISTs is deemed to be the result of tumor metastasis, and therefore indicative of advanced-stage disease. Clinicians need to be aware of these variants, whose prognosis and treatment differ.
authors.affiliation: Department of Pathology, Hospital de Mérida, Mérida, Badajoz, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Díaz-Delgado
authors.forename: A
authors.initials: A
authors.lastname: Hernández-Amate
authors.forename: M
authors.initials: M
authors.lastname: Sánchez-León
authors.forename: S
authors.initials: S
authors.lastname: Pereira-Gallardo
authors.forename: E
authors.initials: E
authors.lastname: Prieto-Sánchez
authors.forename: M
authors.initials: M
authors.lastname: Jiménez-Sáenz
authors.forename: R
authors.initials: R
authors.lastname: González-Cámpora
mesh_terms: D000328:Adult; D002648:Child; D046152:Gastrointestinal Stromal Tumors / Q000235:genetics / Q000473:pathology; D006801:Humans