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Periampullary Cancer
Periampullary cancers are defined as cancers arising within 2 cm of the papilla of Vater. The term “periampullary” includes a heterogenous group of neoplasms arising from the pancreas, duodenum, distal common bile duct, or structures of the ampullary complex ( Fig. 140.1 ). On initial presentation, all these tumors have similar clinical presentation resulting in difficulty in defining the primary site of origin. However, there are fundamental genomic and molecular differences among the four subtypes. The ampullary and the duodenal periampullary tumors have a high estimated 5-year survival (45%–49%), which is better than the bile duct cancer (27%) and pancreatic cancer (18%).
Ampullary Tumors.
The ampulla of Vater is formed by three anatomic components: the ampulla (often formed by a common channel), the intraduodenal portion of the bile duct, and the intraduodenal portion of the pancreatic duct. Among the periampullary tumors, ampullary tumor is the second most common. Ampullary cancers can be divided into intestinal or pancreaticobiliary type based on histomorphology.
Clinical Picture
Patients typically manifest symptoms early in the course of the disease, usually in the seventh decade of life, with abdominal pain, obstructive jaundice, malaise, anorexia, and weight loss. Jaundice is progressive but occasionally may be associated with cholangitis. Iron deficiency anemia as a result of chronic low-grade bleeding is a clinical association. The triad of intermittent painless jaundice, anemia, and enlarged palpable gallbladder (Courvoisier gallbladder) is seen in less than 10% of patients. The stool may be gray or silver as a result of acholic feces mixed with melena. Recurrent acute pancreatitis of no readily identifiable etiology may be the presenting feature.
Risk factors for periampullary tumors are similar to those for pancreatic cancer. Patients with familial adenomatous polyposis are predisposed to ampullary adenomas (see Chapter 100 ).
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