Does Peutz-Jeghers syndrome predispose to gastrointestinal malignancy? A later look

Abstract

Several reports have recently suggested an association between the development of intestinal carcinoma and the presence of Peutz-Jeghers syndrome. During the 45-year period between 1935 and 1979, 48 patients with Peutz-Jeghers syndrome were seen at the Mayo Clinic, Rochester, Minn, and followed up for a median period of 33 years. Carcinoma of the small or large intestine did not develop in any patient, with possibly one exception. Survival of patients with Peutz-Jeghers syndrome also was found to be similar to that of the general population. Therefore, skepticism continues about the premalignant potential of this rare syndrome, especially in the small intestine, and surgical conservatism should be practiced in its management.