Barrett esophagus with progression to adenocarcinoma in multiple family members with attenuated familial polyposis.

Attenuated familial polyposis (AFAP) is a subset of familial adenomatous polyposis (FAP) that has a relatively benign disease course. AFAP is characterized by no more than 100 colorectal polyps and has a tendency toward rectal sparing, a 20–25-year delay in the onset of adenomatosis and bowel symptoms, a 10–15-year delay in the development of colorectal cancer, and death caused by colorectal cancer in 15–20 years. Extracolonic involvement is limited. Although gastric polyps and duodenal adenomas are frequent, esophageal involvement has not been reported in the literature, to our knowledge. We report a case of AFAP with esophageal mucosal disease that progressed from metaplasia to malignancy.