Cronkhite-Canada syndrome
Upper GI endoscopy:
Gastro-esophageal junction nodule.
Pedunculated and sessile polyps throughout stomach and carpet-like polyps in duodenum
Colonoscopy:
Hemorrhagic, ulcerated and nodular rectosigmoid and sigmoid colon.
Histology:
Biopsies from GEJ, stomach, duodenum and sigmoid:
All biopsies showed similar histological features, with no evidence of malignancy. Foveolar hyperplasia was present. Specific features were highly suggestive of Cronkhite-Canada, although a rare variant of juvenile polyposis syndrome could not be entirely excluded microscopically.
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Discussion:
Cronkhite-Canada is a rare hamartomatous polyposis syndrome, affecting the stomach and large bowel, with relative sparing of the esophagus and small bowel. The presence of a GEJ polyp in this case is slightly atypical, but otherwise the clinical features in combination with imaging findings, endoscopy and histology all point to Cronkhite-Canada syndrome as the cause.