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Peutz-Jeghers syndrome

Reviewers: Elliot Weisenberg, M.D. (see Reviewers page)
Revised: 8 August 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.


● Autosomal dominant disorder with mucocutaneous pigmentation and multiple GI hamartomatous polyps, most common in small intestine
● Due to mutation in STKII/LKBI tumor suppressor gene related to TGF-β signalling pathway
● Usually children/teenagers
● Male=female
● 20% of Peutz-Jeghers patients have hamartomatous gastric polyps
● Rarely associated with dysplasia, gastric adenocarcinoma, but may occur at young age (Arch Pathol Lab Med 1982;106:517)

Gross description

● 1-3 cm, short broad stalk, coarsely lobulated

Micro description

● Core of finely arborizing branches of smooth muscle from muscularis mucosa
● Covered by normal but often disorganized mucosa
● Usually no prominent inflammation
● Pseudoinvasion in 10% (no atypia, normal epithelial cell subtypes, brush border, hemosiderin deposition, intramural mucinous cysts, Am J Surg Pathol 1987;11:743)

Micro images

Gastric Peutz-Jeghers polyps

Additional references

Odze RD, Goldblum JR (2009). Surgical Pathology of the GI Tract, Liver, Biliary Tract, and Pancreas. Philadelphia, PA: Saunders
Fenoglio-Preiser CM, Noffsinger AE, Stemmermann GN (2007). Gastrointestinal Pathology: an atlas and text. Philadelphia, PA: Lippincott Williams & Wilkins

End of Stomach > Polyps > Peutz-Jeghers syndrome

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