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Malignancy arising in a jejunal diverticulum is extremely rare. We report a leiomyosarcoma arising from a jejunal diverticulum. This is the second reported case of smooth muscle tumor arising from this site. Despite general belief that small bowel diverticula are devoid of muscle coat, there is presence of a thin muscle coat which can give rise to a neoplasm. We report such a case.
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