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Review
. 2000 Mar;95(3):596-604.
doi: 10.1111/j.1572-0241.2000.01831.x.

Peutz-Jeghers syndrome

T J McGarrity  1 , H E Kulin, R J Zaino
Affiliations
Review

Peutz-Jeghers syndrome

T J McGarrity et al. Am J Gastroenterol. 2000 Mar.

Abstract

Peutz-Jeghers syndrome (PJS) is an unusual polyposis syndrome that has enjoyed a rich and somewhat confusing history. Mucocutaneous pigmentation and diffuse gastrointestinal hamartomas are the hallmark features of this autosomal dominant inherited condition. Peutz-Jeghers syndrome is now also recognized as a cancer predisposition syndrome. In this review, we highlight the historical aspects of PJS polyposis with special emphasis on its extraintestinal manifestations, particularly genital tract tumors. A PJS management scheme for clinicians is included.

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