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8 May 2013 - Case of the Week #272

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Website news:

(1) We have added / updated 11A1, CCR5, CCR8, HPC2, and PLEKHA7 in the Stains chapter.

(2) Our Feature Page for May highlights Computer Software and Systems and includes Milestone Medical, PathView Systems, and Voicebrook. We also have a new Mystery Case on the right side of the Home Page.

(3) In April 2013, we again had record traffic, with 384,599 visits and an average daily traffic of 12,819 visits (324,000 "hits").

(4) For topics with a large number of images, we have started putting them in a scrolling frame to make the space more efficient. For an example, please view CD31 and let us know what you think.

Case of the Week #272

Clinical History:

A 50 year old man had multiple polyps in the jejunum, which were biopsied.

Endoscopic image:



Micro images:




What is your diagnosis?































Diagnosis:

Hamartomatous (Peutz-Jeghers) polyp

Discussion:

Peutz-Jeghers polyps (hamartomatous polyps) are supported by broad bands of muscularis mucosa smooth muscle, which is thicker centrally, and resembles a Christmas tree at low power (see Small bowel chapter). The polyp has superficial columnar and goblet cells, but Paneth and endocrine cells at its base.

Peutz-Jeghers polyps are large, pedunculated polyps of the gut almost always seen in association with Peutz-Jeghers syndrome (Am J Surg Pathol 2007;31:1209). This rare autosomal dominant disorder is usually diagnosed at ages 20-30, with hamartomatous polyps in the small bowel (100%), stomach and colon (25%), and associated adenomatous lesions that may give rise to adenocarcinoma of the stomach, large or small bowel; adenoma malignum of the cervix, ovarian mucinous tumors, and carcinoma of the breast, lung and pancreas. The syndrome is also associated with sex-cord tumor with annular tubules (almost all patients) and melanotic pigmentation of the digits, genitalia, lips, oral mucosa, palms and soles.

Peutz-Jeghers syndrome is caused by mutations in STK11/LKB1, a novel serine threonine kinase (Nat Genet 1998;18:38, Nature 1998;391:184), which may play a role in cell polarity (Genes Cells 2012;17:737).

The differential diagnosis includes invasive carcinoma, because polypoid epithelial misplacement, present in 10%, may resemble malignancy (Am J Surg Pathol 2000;24:34). Recommended treatment is screening and polypectomy of the entire small bowel, plus lifelong cancer screening of the GI tract, breast, lung, ovaries, pancreas and testis (World J Gastroenterol 2009;15:5397), due to the markedly elevated cancer risk, mainly for pancreatic and cervical cancers (Dig Liver Dis 2013 Feb 15 [Epub ahead of print]).

Nat Pernick, M.D., President
and Palak Thakore, Associate Medical Editor
PathologyOutlines.com, Inc.
30100 Telegraph Road, Suite 408
Bingham Farms, Michigan (USA) 48025
Telephone: 248/646-0325
Email: NatPernick@Hotmail.com
Alternate email: NatPernick@gmail.com