A prospective evaluation of the upper gastrointestinal tract and periampullary region in patients with Gardner syndrome

Abstract

Neoplasms of the upper gastrointestinal tract and periampullary region occur in patients with Gardner syndrome (GS), but their true incidence is not yet established. Fourteen patients with GS underwent esophagogastroduodenoscopy prior to colectomy and every 1-3 years thereafter. In 10 patients the pancreatobiliary system was also investigated: nine by endoscopic retrograde cholangiopancreatography and one at autopsy. All of the patients underwent ultrasound examination of liver, biliary tree, and pancreas. Adenomas in the upper gastrointestinal tract were found in all of the patients: Vater's papilla--12, duodenum--nine, and gastric antrum--seven. Only five patients had adenomas at the time of diagnosis of GS, and all the others developed adenomas within a mean of 2.5 years. One patient had hyperplastic polyps in the stomach fundus. Adenomas of the papilla of Vater demonstrated a higher degree of dysplasia compared to dysplasia in other locations of the gastrointestinal tract. Endoscopic retrograde cholangiopancreatography helped in detecting adenomas in distal common bile duct not visible at endoscopy. We conclude that adenomas at the upper gastrointestinal tract occur frequently in patients with GS and, therefore, periodic endoscopic systematic investigation of all patients with GS is mandatory.