Small Intestine Gastrointestinal Stromal Tumors
Small Intestine Gastrointestinal Stromal Tumors
Purpose of review: To review the contemporary management of gastrointestinal stromal tumor (GIST), including endoscopy, surgery, and systemic therapy, highlighting the aspects unique to small intestinal tumors.
Recent findings: Tumor size, mitotic count, and site of origin are the three key prognostic factors, with mitotic count being the single strongest predictor of recurrence. Tumors arising in the small bowel have worse prognosis than those of comparable size and mitotic count arising in other organs. Endoscopy and endoscopic ultrasoundguided, fine-needle aspiration are key components in the diagnosis of GIST. The role of endoscopy in surveillance and resection remain investigational. Surgery, either open or laparoscopic, remains the only curative option, but recurrence rates are high. Adjuvant therapy with imatinib mesylate improves recurrence-free survival rates and may improve overall survival (OS) with longer duration of treatment. Neoadjuvant imatinib may play an important role in the management of patients with locally advanced disease. For patients with advanced disease, first-line imatinib and second-line sunitinib malate have improved progression-free and OS rates. Systemic treatment should be continued life-long or until treatment failure.
Summary: Advances in the last decade have dramatically changed the management and prognosis of patients with primary and advanced GIST.
Gastrointestinal stromal tumor (GIST), although rare, is the most common mesenchymal tumor of the intestinal tract. GISTs were only recognized as a distinct entity in 1983, when it was determined that these tumors contained both smooth muscle and neural features. Subsequently, Hirota et al. recognized that the majority of tumors contained mutations of the KIT proto-oncogene that led to constitutive activation of the KIT receptor tyrosine kinase.
Most of the data on GISTs comes from experience with patients with tumors arising at various sites. This review will discuss the current understanding of GISTs with a specific focus on the recent findings including endoscopic evaluation and management. Issues specific to small bowel GISTs will be highlighted where relevant.
Abstract and Introduction
Abstract
Purpose of review: To review the contemporary management of gastrointestinal stromal tumor (GIST), including endoscopy, surgery, and systemic therapy, highlighting the aspects unique to small intestinal tumors.
Recent findings: Tumor size, mitotic count, and site of origin are the three key prognostic factors, with mitotic count being the single strongest predictor of recurrence. Tumors arising in the small bowel have worse prognosis than those of comparable size and mitotic count arising in other organs. Endoscopy and endoscopic ultrasoundguided, fine-needle aspiration are key components in the diagnosis of GIST. The role of endoscopy in surveillance and resection remain investigational. Surgery, either open or laparoscopic, remains the only curative option, but recurrence rates are high. Adjuvant therapy with imatinib mesylate improves recurrence-free survival rates and may improve overall survival (OS) with longer duration of treatment. Neoadjuvant imatinib may play an important role in the management of patients with locally advanced disease. For patients with advanced disease, first-line imatinib and second-line sunitinib malate have improved progression-free and OS rates. Systemic treatment should be continued life-long or until treatment failure.
Summary: Advances in the last decade have dramatically changed the management and prognosis of patients with primary and advanced GIST.
Introduction
Gastrointestinal stromal tumor (GIST), although rare, is the most common mesenchymal tumor of the intestinal tract. GISTs were only recognized as a distinct entity in 1983, when it was determined that these tumors contained both smooth muscle and neural features. Subsequently, Hirota et al. recognized that the majority of tumors contained mutations of the KIT proto-oncogene that led to constitutive activation of the KIT receptor tyrosine kinase.
Most of the data on GISTs comes from experience with patients with tumors arising at various sites. This review will discuss the current understanding of GISTs with a specific focus on the recent findings including endoscopic evaluation and management. Issues specific to small bowel GISTs will be highlighted where relevant.