BACKGROUBD: Adenomatous polyps and adenocarcinomas of the periampullary region are the most common upper gastrointestinal (UGI) neoplasms encountered in familial adenomatous polyposis (FAP) patients. In addition, tumors arising from the liver, biliary tract, and pancreas have also been reported. The purpose of this study was to review a single institution experience with pancreaticoduodenal surgery for FAP-associated UGI neoplasms.
METHODS: Of the 61 individuals participating in the FAP registry, 8 underwent surgical resection of UGI neoplasms between 1987 and 1998. The charts of these individuals were reviewed for clinical indications, type of surgery, postoperative complications, and outcome.
RESULTS: Of the 8 patients identified, 7 had pancreaticoduodenectomy and 1 had duodenotomy with ampullectomy. The indications for surgery were periampullary cancer (3), severe dysplasia within a duodenal villous tumor (4), and pancreatic cancer (1). At the time of UGI surgery, patients ranged in age from 29-65 and all but one had undergone colorectal surgery, on average 16 years beforehand. Pancreatic ascites after a pylorus-sparing pancreaticoduodenectomy was the only surgical complication. After a mean follow-up of 77 (range 32-156) months, 2 patients have died, neither from their UGI neoplasm. The only patient with evidence of recurrent duodenal adenoma underwent duodenotomy and ampullectomy. Another patient developed confluent jejunal adenomas just beyond the gastroenteric anastomosis almost 12 years after pancreaticoduodenectomy for severe dysplasia of a duodenal villous adenoma.
CONCLUSIONS: Pancreaticoduodenectomy is a safe and appropriate surgical option for FAP patients with villous tumors containing severe dysplasia as well as carcinoma. Postoperative morbidity was minimal and there was no perioperative mortality. This approach not only removes the tumor, but also eliminates the risk of subsequent duodenal cancer. However, adenomas in the small bowel may still develop with prolonged follow-up.