Home   Chapter Home   Jobs   Conferences   Fellowships   Books



Advertisement

Small bowel (small intestine)

Benign tumors and tumor-like conditions

Peutz-Jeghers polyp


Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 8 May 2013, last major update August 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

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 of the Week #272)
● 76 and 84 year old men (J Med Case Rep 2011;5:240)

Gross description
=========================================================================

● Adenomatous-like polyps
● Pedunculated, large, lobulated

Clinical images
=========================================================================



Various images


Endoscopic image

Gross images
=========================================================================



Various images

Micro 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%

Micro images
=========================================================================



Various images

Case of the Week #272:




Virtual slides
=========================================================================



Small intestine, duodenum

Differential diagnosis
=========================================================================

Enteritis cystica profunda
● Invasive carcinoma

End of Small bowel (small intestine) > Benign tumors and tumor-like conditions > Peutz-Jeghers polyp


This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).