The Peutz-Jeghers syndrome is one of the polyposis syndromes. It has an autosomal dominant inheritance and is characterised by :
- multiple hamartomatous polyps, most commonly involving the small intestine, but also colon and stomach
- mucocutaneous pigmentation involving the mouth, fingers and toes
Peutz-Jeghers polyps are hamartomas which have a characteristic feature of a smooth muscle core arising from the muscularis mucosae in a tree-like branching pattern. This distinguishes them from the hamartomatous polyps of Cronkhite-Canada syndrome, juvenile polyposis, and Cowden disease1.
Patients are at increased risk of
- GI tract adenocarcinoma, although the polyps themselves are not premalignant.
There is also increased incidence of extraintestinal malignancy (breast, pancreas, ovary)
The syndrome is named after Jans Peutz (1886 - 1957), a Dutch physician and Harold Jeghers (1904 - 1990), an American physician who had successively described the association between polyposis and the mucocutaneous macules.
- 1. Buck JL, Harned RK, Lichtenstein JE et-al. Peutz-Jeghers syndrome. Radiographics. 1992;12 (2): 365-78. Radiographics (abstract) - Pubmed citation
- 2. Brant WE, Helms CA. Fundamentals of diagnostic radiology. Lippincott Williams & Wilkins. (2007) ISBN:0781761352. Read it at Google Books - Find it at Amazon
Synonyms & Alternative Spellings
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