Abstract

INTRODUCTION: While gastrointestinal stromal tumors (GIST) are among the most common mesenchymal malignancies affecting the gastrointestinal (GI) tract, they are considered rare entities comprising a mere 1-3% of all GI tumors. The vast majority are detected incidentally. We report a case of a small bowel GIST presenting as a severe acute upper GI bleed. CASE DESCRIPTION/METHODS: A 38-year-old female with a history of Henoch Schonlein Purpura presented with melena, lightheadedness, and dyspnea for one week. She was orthostatic on admission and her exam notable for conjunctival pallor and melena in the rectal vault. Her hemoglobin was 7.0 mg/dl on admission with labile measurements throughout her stay. An EGD revealed heme within the gastric body with active oozing in the proximal duodenum but with no identifiable source. Subsequent EGD revealed an actively spurting Dieulafoy lesion in the duodenum. Hemostasis was achieved with epinephrine and deployment of four hemoclips. Hemoglobin levels continued to fluctuate raising concern for re-bleeding. CT angiography of the abdomen was negative for an active bleed, but incidentally demonstrated a 5.8 × 4.7 × 3.7 cm enhancing mass adjacent to the proximal jejunum. She was taken to the OR for diagnostic laparoscopy and underwent en bloc resection of the 4th portion of the duodenum and proximal jejunum. The pathology revealed a low-grade GIST (positive for cKIT proto-oncogene, mitotic index < 5). The patient underwent a follow-up CT nine months after discharge which demonstrated no evidence of recurrent disease. DISCUSSION: Though GISTs are most commonly found in the stomach as well as the small bowel, the duodenum is an uncommon site of proliferation, comprising approximately 3-5% of all cases. Presentation varies, dependent on tumor size, with overt GI bleeding (hematemesis or melena) as the leading complaint in approximately 40-65% of cases. The presence of bleeding is associated with a high probability of tumor necrosis and ulceration and portends a poor prognosis. CT angiography has proven to be an effective imaging modality for the detection of GI bleeds, with sensitivities ranging from 63-90%. Surgical resection is preferred with or without the presence of a bleed. For larger tumors and those with chronic bleeding, oral imatinib is recommended to decrease tumor size. While GISTs commonly present with acute GI bleeding, they are rarely found in the small bowel, making this an important consideration when evaluating acute GI bleeds.

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