Cronkhite-Canada Syndrome
Definition
- Gastrointestinal hamartomatous polyps with associated ectodermal changes
Diagnostic Criteria
- Adult onset non-inherited disorder
- Mean age at onset 60, range 31-86
- Multiple polyps throughout the GI tract
- Esophagus excepted – very rare
- Usually large numbers
- Reported range 5 to innumerable
- Polyps show hamartomatous features
- Essentially indistinguishable from the polyps of Juvenile Polyposis
- Edematous inflamed stroma
- Eosinophils may be prominent
- Irregular, tortuous glands
- Mucus retention cysts in most cases
- Largely intact surface
- Small amounts of smooth muscle may be present in lamina propria
- Most polyps sessile at all sites
- No dysplasia
- Mucosa between polyps typically also shows lamina propria edema and inflammation and mild crypt dilation
- Diagnostic ectodermal findings
- Alopecia
- Onychodystrophy
- Cutaneous hyperpigmented macules
- Most frequent on extremities and face
- Biopsies show increased basal layer pigmentation
- May have increased IgG4 plasma cells
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting: 12/27/09, 11/13/11
