Colon
Syndromes
Cronkhite-Canada syndrome


Topic Completed: 14 April 2021

Minor changes: 14 April 2021

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PubMed Search: Cronkhite Canada syndrome [title] colon


Michael Feely, D.O.
Raul S. Gonzalez, M.D.
Page views in 2020: 1,707
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Cite this page: Feely M, Gonzalez R. Cronkhite-Canada syndrome. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/colontumorcronkhite.html. Accessed April 15th, 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% 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
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 Gonzalez, M.D. and Mike Feely, D.O.

Duodenum: elongated, irregular and cystic crypts; lamina propria has mixed inflammatory infiltrate

Colon: polypoid mucosa with cystically dilated glands and lamina propria expansion



Images hosted on other servers:

Polyp with dilated glands

Duodenum / small intestine

Differential diagnosis
Board review style question #1
    Germline mutation in which of the following genes has been linked to Cronkhite-Canada syndrome?

  1. BRAF
  2. KRAS
  3. None (syndrome is nonhereditary)
  4. TP53
Board review style answer #1
C. None (syndrome is nonhereditary)

Comment Here

Reference: Cronkhite-Canada syndrome
Board review style question #2
    What approximately is the risk of developing carcinoma in patients with Cronkhite-Canada syndrome?

  1. 0 - 5%
  2. 15 - 25%
  3. 40 - 50%
  4. 65 - 75%
Board review style answer #2
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