Biliary neoplasia in Gardner's syndrome

Familial adenomatous polyposis coli is now known to encompass a more complicated spectrum of anomalies than was initially suspected. The term Gardner's syndrome, broadened from its original definition, currently includes all cases of familial polyposis coli with extracolonic neoplasms. Small-bowel adenomata occur in approximately 12% of these patients, and sporadic instances of biliary neoplasia have also been reported. We describe multifocal adenomatous change with severe dysplasia of the gallbladder in a young woman known to have familial adenomatous polyposis coli. Review of this and other such cases in the literature leads us to conclude that neoplasms of the biliary tree constitute a new component of Gardner's syndrome.