GISTs of the large intestine: review of the literature.

Gastrointestinal stromal tumors (GISTs) of the large intestine are extremely rare entities that constitute approximately 5% of the reported GISTs and approximately 0.1% of all cancers of colon and rectum. Almost 85-95% of GISTs contain a mutation in the c-kit tyrosine kinase and positive expression of the CD117 antigen (c-KIT). About 5% of GISTs appear to be c-kit-negative and usually have a mutation on the platelet-derived growth factor receptor-a (PDGFR-a). Compared to other GISTs, GISTs of the colon demonstrate different prevalence, incidence between various population subgroups, clinical appearance, molecular biology, treatment and prognosis. These parameters differ further depending on the GISTs primary site (colon, rectum or anus). The aim of this article was to review the current literature of those rare tumors.