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Gastrointestinal stromal tumors (GISTs) of jejunum is a rare tumor arising from the interstitial cells of Cajal (ICC) present in the myenteric plexus of the muscularis propria in the intestinal wall. They usually present as small asymptomatic mass and a large exophytic mass causing pain is a relatively rare presentation of jejunal GIST. The tumor mass mainly consists of spindle cells arranged in layers or sheets and stain positive for CD117, CD34 and Vimentin on immunohistochemistry. CECT scan is the most common imaging modality used for staging of the tumor. Surgical resection with negative margins is the primary treatment for localized GIST, followed by adjuvant Imatinib in high risk cases. We present the case of a 65 year old male presenting with large extramural jejunal GIST.
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