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Case Rep Gastroenterol. 2009 Apr 10;3(1):49-55.

Duodenal Signet Ring Cell Carcinoma in a Celiac Patient.

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1
Section of General and Thoracic Surgery, Palermo, Italy.

Abstract

Celiac disease results from damage to the small intestinal mucosa due to an inappropriate immune response to a cereal protein. Long-standing or 'refractory' celiac disease is associated with an increased risk of autoimmunity and malignancy. We produced a brief literature review starting from a case of duodenal cancer in a celiac patient. The patient with an history of celiac disease since six months presented with acute manifestation of gastric outlet syndrome. A duodenal stricture was diagnosed at upper gastrointestinal endoscopy and confirmed by abdominal computed tomography. He was successfully treated by segmental duodenal resection. In the resected specimens, the diagnosis was duodenal signet cell adenocarcinoma. 6-month follow-up is uneventful. Primary carcinoma of the duodenum is rare (duodenal adenocarcinoma accounts for less than 0.5% of all gastrointestinal cancers and 30-45% of small intestinal cancers). Some patients with duodenal carcinoma are potentially curable by surgery, but conflicting opinions exist on the factors influencing the survival rate and on surgical treatment as the gold standard. Nevertheless, the goal in surgical treatment is to achieve clear margins. At present, surgical resection (pancreaticoduodenectomy or pancreas-sparing duodenal segmental resection) is the only available option for cure of this disease.

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