Gastrointestinal stromal tumours: Clinical overview, surgery and recent advances in imatinib mesylate therapy

Abstract Aims To review the clinical features of gastrointestinal stromal tumours (GISTs), the role of surgery and its principles and molecular targeted therapies. Methods A Medline-based literature search on relevant topics was performed in PubMed for key articles concerning the clinical features, biology and the novel strategies in the management, whether surgical and/or pharmaceutical, of gastrointestinal stromal tumours. Some information was obtained from Proceedings of the American Society for Clinical Oncology published recently. Results Surgical resection, the first-line intervention for operable GISTs, was historically the only effective treatment. For residual, metastatic and/or inoperable disease, treatment options remain under intense and continuous scrutiny. However, their molecular genetics, i.e. the mutations of the genes coding for KIT or platelet-derived growth factor receptor α, two receptor tyrosine kinases, have been targeted for therapeutic intervention by imatinib mesylate—a tyrosine kinase inhibitor. Conclusion Treatment of GISTs with imatinib has led to dramatic improvements in progression-free and overall survival, thereby rendering its use in the preoperative and postoperative treatment under intense investigation. New investigational agents are being developed and participation in promising clinical trials remains a standard of care.