Periampullary Malignancy in Gardnerʼs Syndrome

Abstract

Three cases of familial polyposis coli [FPC] with associated periampullary malignancies are reported and the literature reviewed, which disclosed 16 additional cases. An additional 5 unreported cases are known to exist. The development of periampullary malignancy in FPC is a definite extracolonic manifestation of the disease and should be considered a variant of Gardner''s syndrome. All FPC patients with colon polyps should undergo routine surveillance of the upper gastrointestinal tract and all duodenal polyps discovered should be surgically removed when feasible.