Multiple Duodenal Adenomas Associated with Familial Adenomatous Polyposis Treated with Pancreas-sparing Total Duodenectomy—A Case Report—

Abstract

A 46-year-old woman underwent total colorectal resection and ileoanal anastomosis at the age of 17 years due to familial adenomatous polyposis (FAP). She also underwent resection for a desmoid tumor at the age of 23, and transanal and endoscopic resections of ileal-pouch polyps at the ages of 32, 36, and 37. At the age of 45 years, she underwent endoscopic submucosal dissection for duodenal lesions of a laterally spreading tumor, which led to the diagnosis of adenocarcinoma (tub1, pTis). Six months later, a 12-mm lesion suspicious for early-stage cancer of type 0-IIa was found on follow-up. The patient was referred to our department for surgical intervention and underwent a pancreas-sparing total duodenectomy (PSTD). Duodenal adenoma is reported to occur in 30-90% of patients with FAP and may become cancerous. Consequently, duodenal adenoma is considered to be the second most important prognostic factor after colorectal cancer. Surgery is considered for duodenal adenomas with Stage IV or higher disease according to the modified Spigelman classification, but there is a lack of evidence and room for debate regarding the choice of surgical technique including PSTD. A review of the literature is presented.