Colon

Syndromes

Cronkhite-Canada syndrome

Authors: Michael Feely, D.O., Raul S. Gonzalez, M.D.

Topic Completed: 14 April 2021

Minor changes: 21 April 2021

Copyright: 2002-2021, PathologyOutlines.com, Inc.

PubMed Search: Cronkhite-Canada syndrome [title] colon

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Table of Contents

Cite this page: Feely M, Gonzalez RS. Cronkhite-Canada syndrome. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/colontumorcronkhite.html. Accessed October 23rd, 2021.

Definition / general

Rare, nonhereditary polyposis syndrome of unknown etiology

Essential features

Disorder of middle adulthood, with mean age of diagnosis of 59 years
Poor prognosis due to associated nutritional complications
Up to 50% of patients have remissions
Rarely has a fulminant course (Endoscopy 2010;42:E350)
Associated carcinomas documented in 15 - 25% of cases (J Clin Pathol 2014;67:891)

Sites

Polyps affect entire gastrointestinal tract, except the esophagus
Also associated with ectodermal changes including alopecia, nail atrophy and cutaneous hyperpigmentation

Pathophysiology

No known genetic link; considered nonhereditary
May represent an autoimmune disorder (Digestion 2007;75:96)

Clinical features

Patients present with variable symptoms, including diarrhea, weight loss, nausea, GI bleeding, protein losing enteropathy

Case reports

17 year old boy with late onset Cronkhite-Canada syndrome (Eur J Gastroenterol Hepatol 2005;17:1139)
54 year old woman with Cronkhite-Canada syndrome associated with myelodysplastic syndrome (World J Gastroenterol 2009;15:5871)
59 year old man with Cronkhite-Canada syndrome complicated with huge intramucosal gastric cancer (Gastric Cancer 2009;12:113)
63 year old woman with colorectal carcinoma in Cronkhite-Canada syndrome (Z Gastroenterol 2001;39:365)
70 year old woman with Cronkhite-Canada syndrome with adenomatous and carcinomatous transformation of colonic polyp (Indian J Gastroenterol 2003;22:189)
72 year old man with Cronkhite-Canada syndrome presenting with adenomatous and inflammatory colon polyps (Nat Rev Gastroenterol Hepatol 2010;7:460)
Acute brain syndrome as a consequence of Cronkhite-Canada syndrome (Psychiatr Danub 2005;17:90)

Clinical images

Images hosted on other servers:

Upper endoscopy: gastric polyposis

Microscopic (histologic) description

Multiple ill defined polyps with cystically dilated glands and crypts associated with an edematous lamina propria containing mononuclear cells and eosinophils, microscopically indistinguishable from juvenile polyps (Am J Surg Pathol 1989;13:940)
Intervening nonpolypoid mucosa characteristically also has dilated glands, edema, congestion and inflammation (Gastroenterol Res Pract 2009;2009:619378)

Microscopic (histologic) images

Contributed by Raul S. Gonzalez, M.D. and Michael Feely, D.O.