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Carcinoma of the Ampulla of Vater

Practice Essentials

Carcinoma of the ampulla of Vater, shown in the image below, is a rare malignant tumor arising within 2 cm of the distal end of the common bile duct, where it passes through the wall of the duodenum and ampullary papilla.

Endoscopic view of an ampullary carcinoma.

Endoscopic view of an ampullary carcinoma.

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The common bile duct merges with the pancreatic duct of Wirsung to form a common channel that exits through the ampulla into the duodenum. The most distal portion of the common bile duct is dilated (ie, forms the ampulla of Vater) and is surrounded by the sphincter of Oddi, which spirals upward around the terminal portion of the duct.

Carcinoma of the ampulla of Vater tends to manifest early due to biliary outflow obstruction, as opposed to pancreatic neoplasms that often are advanced at the time of diagnosis. Clinically, however, ampullary tumors can be virtually indistinguishable from tumors of the distal bile duct or pancreatic head; the point of origin of tumors in this region is typically not determined until the patient undergoes surgery.

Pancreaticoduodenectomy is the standard resection procedure for ampullary carcinoma. When preoperative endoscopic biopsy identifies a lesion as an ampullary adenoma with no high-risk features (eg, high-grade dysplasia), treatment with local resection (ampullectomy) may be considered, if the patient is not a candidate for pancreaticoduodenectomy. However, such patients require surveillance endoscopy to monitor for recurrence.

Surgical resection with curative intent is the only option for long-term survival. Surgical, endoscopic, or radiologic biliary decompression; relief of gastric outlet obstruction; and adequate pain control may improve the quality of life but do not affect overall survival rate.

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