Get the GIST? An overview of gastrointestinal stromal tumours

Abstract

GISTs are the most common mesenchymal neoplasms of the gastrointestinal tract. The last 20 years have been revolutionary in the understanding of these tumours and began with the discovery of c-KIT, a proto oncogene that when mutated forms the molecular basis for the growth and development of these malignancies. Surgery was previously considered to be the only treatment modality in both local and advanced disease, however, the introduction of immunotherapy agents such as tyrosine kinase inhibitors has had profound effects on how we now approach and manage these tumours. These novel agents have significantly reduced the frequency of disease recurrence and dramatically improved survival, and serve as a model for the study of targeted therapies in other solid tumors. We present a review of gastrointestinal stromal tumours and consider the current evidence based detection and management of these unique tumors.

Gastrointestinal Stromal Tumours: A 10 Year Multicenter Audit

“…The most common side effects reported in the literature include fluid retention, GI complaints such as altered bowel habits and nausea, and fatigue. These effects are usually mild however and rarely warrant treatment discontinuation (Watson et al, 2016). Congestive heart failure has been noted in 8.2% of patients, and was reported in two of our patients.…”

“…Mesenchymal tumours are common benign soft tissue neoplasms that may affect the GI tract (Rubin et al, 2000;Watson et al, 2016;Connolly et al, 2003). GISTs represent a subset of this family and may be found in any portion of the GI tract as observed in our patients, however they are most commonly localised to the stomach (60%) and proximal portions of the small intestine (30%) (Rubin et al 2000;Miettinen & Lasota, 2001;Miettinen et al, 1999;Watson et al, 2016).…”

“…Gastrointestinal stromal tumours (GISTs) are rare neoplasms, representing 1% of all primary GI cancers (Rubin et al 2000;Miettinen & Lasota, 2001;Miettinen et al, 1999;Watson et al, 2016). Recent epidemiologic data from Sweden and Iceland report an incidence of 14.5 and 11 per million population, respectively, with a median age of diagnosis 66-69 years (Nilsson et al, 2005;Tryggvason et al, 2005).…”

“…GISTs may be localised to any portion of the gastrointestinal (GI) tract, however they are most often found in the stomach (60%) and proximal portion of the small intestine (30%) (Rubin et al 2000;Miettinen & Lasota, 2001;Miettinen et al, 1999;Watson et al, 2016). Diagnosis is often made incidentally, however patients may complain of nausea, vomiting, abdominal pain and weight loss (Rammohan et al, 2013).…”

“…Advances in our understanding at a molecular level led to the discovery that the mutational activation of the c-KIT oncogene is responsible for stimulating the growth of these cancer cells. This pivotal breakthrough has led to the development of novel treatment agents such as imatinib mesylate (Glivec), resulting in an increased median survival to 60 months (Watson et al, 2016;Otani et al, 2006).…”