PEUTZ-JEGHERS SYNDROME

Peutz-Jeghers syndrome is inherited as an autosomal dominant trait wit h variable incomplete penetrance. Patients with Peutz-Jeghers syndrome characteristically have hamartomatous polyps throughout their entire gastrointestinal tract, particularly in the small bowel, and mucocutan eous hyperpigmentation involving the lips, oral cavity, and skin. Alth ough the intestinal hamartomatous polyps have a lower incidence of mal ignant change in the gastrointestinal tract than do adenomatous polyps , recent information suggests that the overall neoplastic transformati on from Peutz-Jeghers syndrome is not a rare event.