Carcinoma of the Ampulla of Vater

Daniel M Chernoff, MD, PhD
Madeleine D Kraus, MD
Douglass F Adams, MD

April 22, 1994

Presentation

Imaging Findings

Ultrasound examination of the right upper quadrant shows a dilated biliary system (arrow). The right common bile duct measures 11.9 mm in diameter. The common duct is dilated to the level of the ampulla of Vater, where a nonshadowing intraluminal mass (arrow) is visible. The gallbladder contains a small amount of sludge but is otherwise normal. No pancreatic mass is visible.

CT examination confirms the presence of biliary dilatation (arrow) to the distal common bile duct (arrow) with a pancreatic duct of top-normal caliber. Again, no mass is apparent. No enlarged lymph nodes are evident. CT shows a 1 cm indeterminate lesion (arrow) in the left lobe of the liver.

Differential Diagnosis

Diagnosis

Discussion

Primary malignancies of the biliary tract are found in 0.5% of patients undergoing surgery involving the biliary tree. The autopsy frequency of bile duct carcinoma ranges from 0.01 to 0.46%. The usual clinical presentation is painless jaundice; fatigue, pruritis, fever, and nonspecific abdominal pain are also frequently seen.

Options for management of ampullary carcinoma are primarily surgical. The Whipple procedure is the most common surgical approach, with 5-year survival ranging between 18 and 33%. Unresectable cancers are treated by stenting and/or external drainage. External beam radiation and brachytherapy have been attempted with results suggesting a potentially curative role in local disease, but experience is limited.

Pathology

Ampullary carcinoma is the most common primary small bowel malignancy and arises from the epithelium of the pancreatico-biliary tree as it enters the duodenum. Grossly, the tumor may be quite small, and usually has a coarse granular mucosal surface and glistening white cut surface. Microscopically, the tumor is composed of malignant cells that form gland-like structures, the hallmark of adenocarcinoma.

Arrow: distended ampulla.

References

2. Friedman AC, editor. Radiology of the liver, biliary tract, pancreas and spleen. Baltimore: Williams and Wilkins, 1987:802-810.

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