Small intestine & ampulla
Benign tumors / tumor-like conditions
Peutz-Jeghers polyp
Author: Hanni Gulwani, M.B.B.S.
Topic Completed: 1 August 2012
Minor changes: 19 April 2021
Copyright: 2003-2021, PathologyOutlines.com, Inc.
PubMed Search: Peutz-Jeghers polyp[TIAB] small bowel
Table of Contents
Definition / general | Peutz-Jeghers syndrome | Pathophysiology | Clinical features | Case reports | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Differential diagnosisCite this page: Gulwani H. Peutz-Jeghers polyp. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/smallbowelpeutzjeghers.html. Accessed June 19th, 2021.
Definition / general
- Polyp supported by broad bands of muscularis mucosa smooth muscle, thicker centrally, resembling Christmas tree at low power
- Also called hamartomatous polyp
Peutz-Jeghers syndrome
- Autosomal dominant disorder (1 per 200,000) with variable penetrance, usually diagnosed at age 20 - 30
- Hamartomatous polyps in small bowel (100%), stomach and colon (25%) - polyps may occur without other features of syndrome, may be associated with enteritis cystica profunda, may cause intussusception and bleeding
- Polyps are benign but adenocarcinoma may arise from associated adenomatous lesions
- Melanotic pigmentation of digits, genitalia, lips, oral mucosa, palms, soles
- Also sex cord tumor with annular tubules (almost all patients); adenocarcinomas of stomach, large or small bowel; adenoma malignum of cervix, ovarian mucinous tumors, carcinomas of breast, lung, pancreas, uterus
Pathophysiology
- Aberrant intestinal stem cell lineage dynamics in Peutz-Jeghers syndrome leads to protracted clonal evolution in the crypt (Gut 2012;61:839)
Clinical features
- Sporadic Peutz-Jeghers polyp have a cumulative lifetime risk of cancer similar to those with syndrome (Am J Surg Pathol 2007;31:1209)
- Large polyps (> 15 mm) may cause intussusception and GI bleeding (Am J Gastroenterol 2011;106:940)
- Misplaced dysplastic epithelium may resemble malignancy (Am J Surg Pathol 2000;24:34)
Case reports
- 13 year old girl with solitary ileal polyp (Case Rep Gastroenterol 2010;4:452)
- 50 year old man with multiple polyps in jejunum (Case #272)
- 76 and 84 year old men (J Med Case Rep 2011;5:240)
Clinical images
Case #272
Endoscopic image
Images hosted on other servers:
Various images
Gross description
- Adenomatous-like polyps
- Pedunculated, large, lobulated
Gross images
Images hosted on other servers:
Various images
Microscopic (histologic) description
- Polyp supported by broad bands of muscularis mucosa smooth muscle, thicker centrally
- Christmas tree appearance at low power
- Columnar and goblet cells superficially
- Paneth and endocrine cells at base
- Epithelial misplacement (pseudoinvasion) in 10%
Microscopic (histologic) images
Case #272
Images hosted on other servers:
Various images
Differential diagnosis
- Enteritis cystica profunda
- Invasive carcinoma
