Periampullary tumors are neoplasms that arise in the vicinity of the ampulla of Vater. Neoplasms that arise in this site can originate from the pancreas, duodenum, distal common bile duct (CBD), or the structures of the ampullary 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 confluence of the distal common bile duct and the pancreatic duct (figure 2).
It can be difficult to distinguish a primary ampullary carcinoma from other periampullary tumors preoperatively. However, true ampullary cancers have a better prognosis than periampullary malignancies of pancreatic or bile duct origin. Resectability rates are higher, and five-year survival rates are approximately 30 to 50 percent in patients with limited lymph node involvement. In contrast, fewer than 10 percent of patients with completely resected node-positive pancreatic cancer are alive at two years. Thus, an aggressive approach to diagnosis and treatment of periampullary tumors is needed to ensure that patients with these comparatively favorable cancers are treated optimally.
This topic review will cover the treatment and prognosis of ampullary carcinomas. The epidemiology, biologic behavior, clinical manifestations, diagnosis and staging are covered separately. (See "Ampullary carcinoma: Epidemiology, clinical manifestations, diagnosis and staging".)
TREATMENT FOR LOCALIZED DISEASE
The only potentially curative treatment for ampullary carcinoma is surgical resection. Complete tumor resection with negative margins (R0 resection) is a prerequisite for cure.