Familial adenomatous polyposis

Abstract

Duodenal and periampullary cancer is the most common cause of cancer death in patients with familial adenomatous polyposis who have undergone colectomy. Endoscopic surveillance of upper gastrointestinal adenomas is recommended for patients with familial adenomatous polyposis but the timing and appropriate treatment of neoplasms is unknown. The purpose of this experiment was to report our experience with endoscopic and surgical treatment of advanced duodenal adenomas in patients with familial adenomatous polyposis. The records of all patients with familial adenomatous polyposis who had undergone surgical or endoscopic treatment for duodenal adenomas were identified. Data including endoscopic surveillance findings, type of intervention, pathology, and follow-up of the lesions were reviewed. Ten neoplasms >1 cm were treated in eight patients (mean age at the time of diagnosis was 49 years). Nine lesions were histologically advanced. Five lesions involved the papilla. Endoscopic treatment was performed for six lesions. Four lesions recurred, and three were then treated surgically. Local resection was performed for five lesions. Four lesions recurred and two had further operative intervention. Pancreas-sparing duodenectomy was performed in three patients. At a mean follow-up period of 45.7 months, there has been no recurrence. Endoscopic eradication is an appropriate initial treatment for histologically advanced, noncancerous neoplasms or for patients who are not surgical candidates. Pancreas-sparing duodenectomy may be the treatment of choice for patients with carcinoma and those who have failed endoscopic therapy.