Gastrointestinal stromal tumours: An update

The recognition of the central role played by activating mutations of c-kit and platelet derived growth factor receptor alpha in the pathogenesis of GISTs and the introduction of STI-571, a receptor tyrosine kinase inhibitor that has proved extremely effective in the treatment of GISTs, have not only have focused attention on these tumours but also made it necessary to accurately define these tumours and separate them from other mesenchymal lesions. This review discusses the definition, histogenesis, epidemiology, clinical presentation, morphological and immunohistochemical features and the differential diagnosis of GISTs and addresses predictors of malignancy and management of GISTs.