April 1969

The Peutz-Jeghers SyndromeIs There a Predisposition to the Development of Intestinal Malignancy?

Author Affiliations

Rochester, Minn
From the sections of surgery (Dr. Judd), surgical pathology (Dr. Dahlin), and medicine (Dr. Bartholomew), Mayo Clinic and Mayo Foundation and the Mayo Graduate School of Medicine (University of Minnesota), Rochester (Dr. Dozois).

Arch Surg. 1969;98(4):509-517. doi:10.1001/archsurg.1969.01340100125017

In 1921 in Holland, Peutz1 first reported this interesting condition. Later, more elaborate attention was given to the syndrome by Jeghers,2 and finally, in 1954, Bruwer et al3 bestowed the title of "Peutz-Jeghers syndrome" on this unusual clinical condition of mucocutaneous melanin pigmentation with coincidental intestinal polyposis.

Earlier authors1,2,4 considered the polyps to be adenomatous, implying a possible precancerous condition. Approximately 20% of the case reports contained descriptions of malignant changes within the polyps. Later, other investigators5-7 felt that this high incidence of malignancy resulted from misinterpretation of the peculiar nature of these polyps, which they defined as "hamartomas." This interpretation has now gained general acceptance and has been confirmed recently by electron microscopy.8 Isolated reports of apparent gastrointestinal tract malignancies in patients with the PeutzJeghers syndrome have stimulated further thought on the interpretation of evidence and, therefore, the proper treatment of the patients.

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