Small bowel (small intestine) - Peutz-Jeghers polyp

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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
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● 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:
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● 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
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● Aberrant intestinal stem cell lineage dynamics in Peutz-Jeghers syndrome leads to protracted clonal evolution in the crypt (Gut 2012;61:839)

Clinical features:
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● 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
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● 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
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● Adenomatous-like polyps
● Pedunculated, large, lobulated

Clinical images
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Various images

Endoscopic image

Gross images
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Various images

Micro description
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● 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
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Various images

Case of the Week #272:

Virtual slides
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Small intestine, duodenum

Differential diagnosis
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● Enteritis cystica profunda
● Invasive carcinoma

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

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