Young Male Presenting With Massive Hematemesis … Getting to the GIST of It

Abstract

A 36-year old white male with no prior medical history presented with massive hematemesis and melena for one day. He endorsed lightheadedness and fatigue. Emergent esophagogastroduodenoscopy revealed a large 3 cm subepithelial, ulcerated bleeding mass in the second portion of the duodenum, distal to the major papilla (Image 1, 2). Hemostatic clipping was unsuccessful, causing more bleeding. Despite subsequent epinephrine injection, the mass continued to ooze at the end of procedure (Image 3). Interventional radiology performed a visceral arteriogram which showed no evidence of contrast extravasation; embolization was not attempted. Due to ongoing bleeding, urgent surgery with duodenotomy and excision of the mass was performed. Pathology revealed gastrointestinal stromal tumor (GIST) with zero mitoses, and reactive DOG1 and CD117 stains. He was discharged on post-operative day 5 with plans for outpatient oncology follow up. GISTs represent a rare cause of upper GI bleeds. The yearly incidence of GIST is 0.78/100,000, with only 7.5% occurring in the duodenum. GISTs are male predominate, more common in black patients, and have an average age of discovery of 63. Duodenal GISTs typically present with GI bleeding, abdominal pain, or discomfort, and are most frequently found in the second portion of duodenum. Treatment of localized duodenal GISTs without prior analysis of tissue (thus excluding from imatinib pre-treatment) is complete surgical resection. In low risk patients such as ours (no mitotic cells), post-operative imatinib is not required, and the patients only require post-treatment surveillance. Current National Comprehensive Cancer Network recommendations are a CT scan every 3-6 months for 3-5 years, and then annually. Studies have shown only a high mitotic rate as a predictive factor for mortality, as opposed to tumor size or grade. The rate of recurrence free survival is reported as 95% at one year, and 86.7% by 3 years. Even with its low malignant potential, patients with duodenal GISTs should maintain long-term follow up to avoid late relapse.Figure: Duodenal GIST.Figure: Bleeding Duodenal GIST.Figure: Continuous Bleeding of Duodenal GIST.