Table of Contents
Definition / general | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Additional referencesCite this page: Weisenberg E. Peutz-Jeghers. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/stomachpeutzjeghers.html. Accessed March 31st, 2025.
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)