Surveillance and Treatment of Periampullary and Duodenal Adenomas in Familial Adenomatous Polyposis.

Patients with familial adenomatous polyposis (FAP) have a cumulative lifetime risk of over 90% for developing duodenal adenomas, which are the precursor lesions for duodenal adenocarcinoma. Consequently, these patients have a 5% to 10% lifetime risk of periampullary or duodenal adenocarcinoma, making this the leading cause of cancer death in FAP patients who have had prophylactic colectomies. The increased relative risk of duodenal carcinoma in FAP patients and the poor outcomes associated with the treatment of advanced duodenal cancer have led to the development of prevention strategies for this cancer in the setting of FAP. It is generally accepted that surveillance for duodenal adenomas and adenocarcinomas should be included in the management of patients with FAP, although there are few data from clinical trials that demonstrate the effectiveness of surveillance strategies or chemoprevention for the prevention of death from duodenal cancer. Prospective case series have shown that endoscopic surveillance with endoscopic or surgical treatment of high-risk lesions in the duodenal or periampullary region can be performed with successful removal of the at-risk lesion(s). Surveillance should begin at about 21 years of age and should be performed using both an end-viewing and a side-viewing upper endoscope. An interval of 3 to 5 years between examinations appears to be adequate if no polyposis is evident. Once polyposis develops, an interval of 1 to 3 years between screenings for mild polyposis is appropriate. Patients with denser polyposis or larger adenomas are recommended to undergo examination every 6 to 12 months because of their increased risk of developing duodenal adenocarcinoma. Nonsteroidal anti-inflammatory drug therapy with sulindac, a nonselective cyclooxygenase (COX) inhibitor, or celecoxib, a COX-2 selective inhibitor, may be of benefit after the development of duodenal polyposis by inducing the regression or stabilization of the polyposis, although there is limited evidence from randomized, controlled trials to support its routine use. Almost all cases of adenocarcinoma occur in patients with advanced polyposis (Spigelman stage IV disease), and approximately 33% of this group will go on to develop adenocarcinoma if left untreated. The most definitive procedure for reducing the risk of adenocarcinoma is surgical resection of the ampulla and/or duodenum. Pancreaticoduodenectomy or pancreas-sparing duodenectomy are appropriate surgical therapies that are believed to substantially reduce the risk of developing periampullary adenocarcinoma. However, these procedures are associated with significant morbidity and mortality, including the risk of inducing desmoid tumor formation in FAP patients.