Multiple non-metastatic gastrointestinal stromal tumors: Differential features

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.

Bibliographic Details

Main Authors:	Díaz Delgado,M., Hernández Amate,A., Sánchez León,M., Pereira Gallardo,S., Prieto Sánchez,E., Jiménez Sáenz,M., González-Cámpora,R.
Format:	Digital revista
Language:	English
Published:	Sociedad Española de Patología Digestiva 2010
Online Access:	http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082010000800006
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