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Carcinoid tumors of the extrahepatic bile ducts are exceedingly rare, accounting for between 0.1 and 2% of all gastrointestinal carcinoid tumors, with most reported cases arising from the gallbladder. Only 34 cases of carcinoid tumor arising from the extrahepatic bile duct have been documented in the world literature. We report one case of a carcinoid tumor of the common bile duct. A 67-year-old woman sought treatment for obstructive jaundice, accompanied by epigastric pain. The laboratory and imaging studies were consistent with a malignant obstruction in the common bile duct. We performed a Pylorus preserving pancreaticoduodenectomy. Pathologically, an ill-demarcated mass, measuring 1.6x1.5x0.5 cm in size, was noted in the common bile duct, with infiltration to the adjacent pancreatic tissues. Immunohistochemically, the mass was shown to be chromogranin, synaptophysin and CD56 positive. The final pathological diagnosis was one of a well- differentiated carcinoid tumor of a malignant nature. The patient, who underwent a curative surgical resection, was alive and disease free at time of this published report.
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