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Updated: Jun 10, 2026
Molecular and Immunologic Techniques in a Genetically Engineered Mouse Model of Gastrointestinal Stromal Tumor
Published on: May 2, 2022
Multiple non-metastatic gastrointestinal stromal tumors. Differential features
M Díaz-Delgado1, A Hernández-Amate, M Sánchez-León
1Department of Pathology, Hospital de Mérida, Mérida, Badajoz, Spain.
Introduction:
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.
Sources:
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.