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Colon tumor

Familial polyposis syndromes of colon

Cronkhite-Canada syndrome


Reviewers: Charanjeet Singh, M.D. (see Reviewers page)
Revised: 14 October 2011, last major update August 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

Clinical features
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● Rare, non-hereditary disorder of multiple GI juvenile type polyps, usually colonic
● Associated with ectodermal changes (alopecia, nail atrophy and hyperpigmentation), protein losing enteropathy with associated diarrhea, weight loss, abdominal pain, weakness and anorexia
● Polyps affect stomach, colorectum and small intestine
● Usually age 50-60 years
● Poor prognosis due to GI polyposis and nutritional complications; up to 50% may have remissions, can rarely have a fulminant course (Endoscopy 2010;42 Suppl 2:E350)
● 15% develop colon carcinoma (Surg Today 1997;27:345)
Genetics: no known mutations, not familial (unlike juvenile polyposis)

Clinical images
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Endoscopy

Case reports
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● 17 year old boy (Eur J Gastroenterol Hepatol 2005;17:1139)
● 54 year old woman with myelodysplastic syndrome (World J Gastroenterol 2009;15:5871)
● 63 year old woman with p53 mutations (Z Gastroenterol 2001;39:365)
● 63 year old man with use of nuclear medicine scan to locate areas of protein losing enteropathy (Dis Colon Rectum 2005;48:870)
● 70 year old woman with adenoma containing focus of carcinoma (Indian J Gastroenterol 2003;22:189)
● 72 year old man (Nat Rev Gastroenterol Hepatol 2010;7:460)
● Acute brain syndrome due to malabsorption (Psychiatr Danub 2005;17:90)

Micro description
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● Multiple juvenile-type polyps with broad sessile base, cystically dilated glands filled with proteinaceous fluid or inspissated mucus and expanded edematous lamina propria (Am J Gastroenterol 1988;83:772)
● Often are serrated adenomas (Digestion 2004;69:57)
● May have surface erosions and eosinophilic infiltrate
● Typically no dysplasia (Am J Surg Pathol 1989;13:940)
● Mucosa between polyps also has dilated glands, edema, congestion and inflammation (Gastroenterol Res Pract 2009;2009:619378)

Micro images
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Duodenum / small intestine:

Elongated, irregular and cystic crypts lined by attenuated epithelium; lamina propria has mixed inflammatory cell infiltrate


Focal hyperplastic features with dilated glands

Additional references
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eMedicine #1; #2

End of Colon tumor > Familial polyposis syndromes of colon > Cronkhite-Canada syndrome


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