Periampullary malignancy in Gardner's syndrome.

Three cases of familial polyposis coli with associated periampullary malignancies are reported and the literature reviewed, which disclosed 16 additional cases. An additional five unreported cases are known to exist. The authors believe that 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. It is recommended that all FPC patients with colon polyps undergo routine surveillance of the upper gastrointestinal tract and that all duodenal polyps discovered be surgically removed when feasible.