Gastrointestinal Stromal Tumors: An Update for the General Surgeon

Abstract

Gastrointestinal stromal tumors (GIST) were recognized as a specific entity in the late 1980s based on clinical, histopathological and immunohistochemical features and were thought to be derived from smooth muscle cells given the spindle cell appearance at light microscopy. In 1998, Hirota and colleagues achieved a significant breakthrough by demonstrating a near-universal expression of the transmembrane KIT receptor tyrosine kinase (c-KIT, CD 117, stem cell growth factor receptor) on the surface of GIST, while other spindle cell tumours are typically c-KIT negative. Currently, GIST are thought to arise from pacemakers cells, the interstitial cells of Cajal, as both express c-KIT protein.

Ground-breaking understanding of molecular mechanisms involved in molecular pathophysiology of GIST has led to improved overall survival of patients by targeting specific cellular signalling pathways. The practicing general surgeon will need an understanding of the specific diagnostic and therapeutic options for these patients. The treatment of metastatic GIST patients with oral inhibitor molecule of KIT has improved median survival from 18 months to over 5 years. This has led to a growing cohort of patients surviving GIST over many years who will be managed in general surgical practice.