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PubMed Search: Peutz-Jeghers syndrome

Elliot Weisenberg, M.D.
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Cite this page: Weisenberg E. Peutz-Jeghers. website. Accessed September 30th, 2023.
Definition / general
  • 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
Microscopic (histologic) 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)
Microscopic (histologic) images

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Gastric Peutz-Jeghers polyps

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