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Spectrum of extracolonic gastrointestinal tract involvement in Gardner's syndrome.

To define the prevalence of extracolonic gastrointestinal tract neoplasms in association with Gardner's syndrome, 17 patients with the syndrome underwent a systematic endoscopic and radiologic investigation. Adenomas were found at one or more sites in the upper gastrointestinal tract in all patients, most frequently at the papilla of Vater (64.7% of patients). Focal adenomatous changes in normal-appearing mucosa were seen in five patients, at the ampulla of Vater and in the duodenum in four, and in the gastric antrum in two patients. Adenomas were also seen in the duodenum (47%), stomach (29.4%) and small intestine (23.5%). Hyperplastic polyps were found in the gastric antrum, duodenum or small bowel in five patients. Adenomas of the papilla of Vater showed greater degrees of dysplasia than did adenomas of the stomach or small bowel, which might explain the more frequent occurrence of malignancy at the papilla of Vater in patients with Gardner's syndrome. A systematic investigation of the gastrointestinal tract is mandatory in all patients with Gardner's syndrome prior to surgery, and at periodic follow-up thereafter.