Gastrointestinal stromal tumour: From the clinic to the molecules

GastroIntestional stromal tumours (GISTs), the most frequent sarcoma in the gastro-intestinal (GI) tract, are highly resistant to conventional chemotherapy and radiotherapy. These tumours have activating mutations in two closely related genes, KIT (75-80%) or/and PDGFRA (5-10%). Targeting these mutated activated proteins with imatinib mesylate has proven efficient in the treatment of GISTs. The median survival after diagnosis of GIST increased from 1.5 to 4.8 years with imatinib treatment. However, resistance to imatinib eventually develops and new-targeted therapies are needed. This paper reviews the medical, clinical and pathological aspects of GISTs based on latest research in human cell lines and animal models