Gastric adenocarcinoma associated with fundic gland polyps in a patient with attenuated familial adenomatous polyposis

Familial adenomatous polyposis (FAP) is a rare autosomal dominant precancer ous condition of the colon caused by mutations in the adenomatous polyposis coli (APC) tumor suppressor gene. FAP is characterized by the appearance o f innumerable adenomatous polyps throughout the large bowel. Fundic gland p olyps are the most common gastric lesion in FAP. It is generally believed t hat fundic gland polyps have little or no potential for malignant transform ation in the population at large, and only a few case reports describe the development of high grade dysplasia or gastric adenocarcinoma associated wi th diffuse fundic gland polyposis in patients with FAP. We report the secon d case of gastric adenocarcinoma intimately associated with fundic gland po lyposis in a family with an attenuated form of FAP. The patient had undergo ne routine screening per current guidelines because of his known mutation i n the APC gene. This suggests that malignant transformation of fundic gland polyps in patients with FAP occur more frequently than previously believed . Current screening recommendations may not be sufficient for patients with FAP or its attenuated forms. (Am J Gastroenterol 1999;94:2275-2281. (C) 19 99 by Am. Coll. of Gastroenterology).