Multiple non-metastatic gastrointestinal stromal tumors. Differential features.
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.
Díaz-Delgado
M
M
Department of Pathology, Hospital de Mérida, Mérida, Badajoz, Spain.
Hernández-Amate
A
A
Sánchez-León
M
M
Pereira-Gallardo
S
S
Prieto-Sánchez
E
E
Jiménez-Sáenz
M
M
González-Cámpora
R
R
D000328:Adult; D002648:Child; D046152:Gastrointestinal Stromal Tumors / Q000235:genetics / Q000473:pathology; D006801:Humans