| Theme |
Gastrointestinal polyposis -- progress in the diagnosis and treatment |
| Title |
Cronkhite-Canada syndrome |
| Author |
Chikako Watanabe |
Department of Internal Medicine, National Defense Medical College |
| Author |
Soichiro Miura |
Department of Internal Medicine, National Defense Medical College |
| Author |
Ryota Hokari |
Department of Internal Medicine, National Defense Medical College |
| [ Summary ] |
Cronkhite-Canada syndrome (CCS) is a rare, acquired gastrointestinal polyposis syndrome with dermatologic manifestations. Patients with chronic active CCS polyposis have a higher risk of developing GI cancer than the general population. Appropriate medical therapy and endoscopic surveillance can alter the natural history of CCS. Oral corticosteroid therapy was confirmed to be effective for active CCS. Its benefits, including clinical improvement and polyp regression are usually apparent within 12 months. Adjunctive nutritional therapy may decrease the overall duration of corticosteroid therapy. Durable polyp regression (endoscopic remission) is associated with a markedly improved prognosis with decreased cancer risk. To prevent the risk of cancer development, surveillance endoscopy is recommended to assess mucosal disease activity and to remove adenomas and other premalignant mucosal lesions, although no long-term surveillance data are available. |