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Ampullary carcinoma: Epidemiology, clinical manifestations, diagnosis and staging

John A Martin, MD
Section Editor
Douglas A Howell, MD, FASGE, FACG
Deputy Editors
Diane MF Savarese, MD
Shilpa Grover, MD, MPH, AGAF


Periampullary tumors are neoplasms that arise in the vicinity of the ampulla of Vater. They can originate from the pancreas, duodenum, distal common bile duct (CBD), or the structures of the ampullary (ampulla of Vater) complex. The ampulla of Vater is formed by the duodenal aspect of the sphincter of Oddi muscle, which surrounds the confluence of the distal CBD and main pancreatic duct, as well as the papilla of Vater, a mucosal papillary mound at the distal insertion of these ducts on the medial wall of the duodenum (figure 1). Ampullary carcinomas are defined as those that arise within the ampullary complex, distal to the bifurcation of the distal CBD and the pancreatic duct (figure 2).

The papilla is a nipple-like structure on the medial aspect of the second portion of the duodenum best visualized with a side-viewing endoscope. The distal bile and ventral pancreatic ducts traverse the duodenal wall in this location and open into the duodenal lumen through the small mucosal elevation of the papilla of Vater.

The epidemiology, clinical features, diagnosis, and staging of ampullary carcinoma will be reviewed here. Treatment of ampullary cancers and the approach to the patient with ampullary adenoma are presented separately. (See "Ampullary carcinoma: Treatment and prognosis" and "Ampullary adenomas: Clinical manifestations and diagnosis" and "Ampullary adenomas: Management".)


Neoplastic transformation of the intestinal mucosa occurs more commonly near the ampulla than at any other site in the small intestine. Despite this, primary ampullary tumors are rare, with an incidence of approximately four to six cases per million population [1-4]. They account for only 6 percent of lesions that arise in the periampullary region [5] but are responsible for 20 percent of all tumor-related obstructions of the common bile duct [6]. There is some evidence that the incidence has increased over the last 30 years [3].

Both benign and malignant ampullary tumors can occur sporadically or in the setting of a genetic syndrome. The incidence of ampullary tumors is increased 200- to 300-fold among patients with hereditary polyposis syndromes, such as familial adenomatous polyposis (FAP) and hereditary nonpolyposis colorectal cancer (HNPCC), compared with the general population [7-9]. Surveillance endoscopy is particularly important to detect early ampullary lesions in patients with FAP given the high incidence of coexisting premalignant duodenal adenomatous polyps. Up to 90 percent of patients with FAP develop adenomas in the upper gastrointestinal tract [10]. (See "Familial adenomatous polyposis: Screening and management of patients and families".)

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Literature review current through: Nov 2017. | This topic last updated: Mar 23, 2017.
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