Peutz-Jeghers syndrome.
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.
McGarrity
T J
TJ
Department of Medicine, The Milton S. Hershey Medical Center, The Pennsylvania State University College of Medicine, Hershey 17033-0850, USA.
Kulin
H E
HE
Zaino
R J
RJ
D011125:Adenomatous Polyposis Coli / Q000175:diagnosis* / Q000235:genetics; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D008297:Male; D010580:Peutz-Jeghers Syndrome / Q000175:diagnosis* / Q000235:genetics; D011230:Precancerous Conditions / Q000175:diagnosis* / Q000235:genetics; D012307:Risk Factors