Carcinoma and polyps of the gallbladder associated with Peutz-Jeghers syndrome.

Peutz-Jeghers syndrome is a genetic condition characterized by mucocutaneous pigmentation and gastrointestinal polyposis. A variety of neoplasms have been found in the alimentary tract or elsewhere in patients with this entity. A 39-year-old female patient who had carcinoma and two polyps in the gallbladder in association with Peutz-Jeghers syndrome is described. Since the polyps consisted of the normal lining epithelium of the gallbladder and pseudopyloric gland-type metaplastic cells and obviously lacked cellular atypism, the authors would consider them hamartomatous. The carcinoma partly showing submucosal invasion existed in an area other than the polyps. This is the first documented case of the syndrome having gallbladder carcinoma.