Does Peutz-Jeghers syndrome predispose to gastrointestinal malignancy? A later look
D. A. Linos, R. R. Dozois, D. C. Dahlin and L. G. Bartholomew
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.
