The first ampullary resection was performed by Halsted in 1897 by resecting part of the ampulla and reimplanting the common bile duct and pancreatic duct back in the duodenum. Tumors of the ampulla of Vater are rare lesions that comprise less than 10% of pancreatic and periampullary cancers. Benign tumors include lipomas, hamartomas, lymphangiomas, hemangiomas, leiomyofibromas, and neurogenic tumors. Most malignant tumors are adenocarcinomas but adenosquamous carcinoma, leiomyosarcoma, fibrosarcoma, neuroendocrine carcinomas are also found. Patients typically present at an earlier stage than tumors of the pancreatic head or tail or the distal common bile duct. The most common symptom is jaundice, though this is more frequently seen in adenocarcinoma than adenomas. Other common symptoms include fever, weight loss, nausea, pruritus, pancreatitis (more common in adenomas), abdominal pain, and anemia secondary to bleeding.

Controversy exists regarding the procedure of choice for ampullary masses. Surgical options for treating ampullary tumors include endoscopic removal, transduodenal ampullectomy, or pancreaticoduodenectomy (PD). In patients with sporadic ampullary polyps, all should undergo attempts at resection when medically possible. Endoscopic removal is an alternative to surgery that has become technically more feasible. Most ampullary neoplasms that are 2 cm or less and are confined to the superficial layers of the duodenum can be resected endoscopically by experienced practioners. Relative contraindications to endoscopic resection, and indications for surgical resection, include large lesions, recurrence after endoscopic resection, lesions in a duodenal diverticulum, penetration into the duodenal wall or along the bile duct, inability to access the duodenum endoscopically, and multiple polyps.

Several authors have recommended surgical ampullectomies for benign lesions less than 3 cm, small neuroendocrine tumors, and T1 carcinomas of the ampulla. Surgical ampullectomy is contraindicated in those with lesions so large and irregular that reconstruction is not possible. If frozen section is free of cancer, but carcinoma is found on final pathologic examination, the patient should be considered for interval pancreaticoduodenectomy. Pancreaticoduodenectomy is performed when (1) invasive carcinoma is found on preoperative biopsy, (2) margins are not safely obtainable, (3) frozen section shows carcinoma, and (4) histologic confirmation of cancer is not possible on selected patients with ampullary obstruction.

The first case of duodenal carcinoma was described by Hamburger in 1746. Duodenal carcinoma represents about 0.3% of all malignant neoplasms of the gastrointestinal tract and 25% to 45% of malignant neoplasms of the small intestine. Patients may present with obstructive symptoms, jaundice, abdominal pain, anorexia, or bleeding. Most duodenal cancers are preceded by premalignant polyps in the duodenum. Adenomatous tissue has been found within duodenal cancer, suggesting the existence of an adenoma-carcinoma sequence in the duodenum. Polyps may be sporadic or in conjunction with polyposis syndromes, such as familial adenomatous polyposis (FAP), Gardner’s, and Peutz-Jeghers. These patients are at increased risk for duodenal cancer. After colectomy, this accounts for the majority of cancer-related deaths. Duodenal polyposis is staged according to polyp number, polyp size, histologic type, and dysplasia (Table 16.1).Although 95% of polyposis patients have adenomatous polyps in the duodenum, only 10% have severe or stage IV duodenal polyposis, and only 5% go on to develop cancer. These patients should undergo regular endoscopic examinations. Those who are unable to undergo surveillance, or whom have rapid polyp growth or severe dysplasia, should undergo either pancreaticoduodenectomy or pancreas-sparing duodenectomy (PSD) since the entire duodenal mucosa is at risk. Pancreaticoduodenectomy, however, carries a morbidity and mortality that are too high for dealing with a premalignant condition. In patients with FAP or other polyposis syndromes, endoscopic removal is impractical, as there are typically multiple polyps, most are sessile, and they frequently cluster around the ampulla of Vater. Local excision of polyps is not sufficient surgical treatment, as morbidity is high and recurrence is expected. Contraindications to pancreas-preserving duodenectomy include invasive cancer in the duodenum or neoplasm that tracks up the bile duct outside the confines of the duodenum.