FaCD Online Syndrome Fact Sheet

Last updated: 01 Jul 2009

Name: Cronkhite-Canada syndrome

Mode of Inheritance: multifact?/ spor?

Tumor features

gastrointestinal hamartomas
gastrointestinal polyps, juvenile type
serrated adenomas, colorectal

Tumor features (possible)

gastrointestinal cancer
multiple myeloma (Kahler's disease)

Non-tumor features

alopecia
hyperpigmentation of the skin
nails, dystrophic

Comment

This disorder is characterized by hamartous (juvenile type) gastrointestinal polyposis associated with diarrhea, alopecia, skin hyperpigmentation (extremities, face, palms & soles, and neck are the sites most frequently affected) and dystrophic nails. All cases are sporadic and have an adult-age onset. Gastrointestinal cancer (including multiple gastric cancer[1]) has been reported in this disorder. [2-5]. Serrated adenomas associated with colon cancer have been reported in this syndrome as well[6].

References

[1] Watanabe T, Kudo M, Shirane H, Kashida H, Tomita S, Orino A, Todo A, Chiba T. Cronkhite-Canada syndrome associated with triple gastric cancers: a case report. Gastrointest Endosc 1999; 50(5):688-691.
[2] Daniel ES, Ludwig SL, Lewin KJ, Ruprecht RM, Rajacich GM, Schwabe AD. The Cronkhite-Canada Syndrome. An analysis of clinical and pathologic features and therapy in 55 patients. Medicine Baltimore 1982; 61(5):293-309.
[3] Goto A. Cronkhite-Canada syndrome: epidemiological study of 110 cases reported in Japan. Nippon Geka Hokan 1995; 64(1):3-14.
[4] Bacher T, Schonekas H, Steurer KT, Wunsch PH. The Cronkhite-Canada syndrome, a rare cause of gastrointestinal polyposis. Dtsch Med Wochenschr 122[21], 676-681. 1997.
[5] Karasawa H, Miura K, Ishida K, Sase T, Kobayashi T, Kinouchi M, Okabe M, Ando T, Kaneko N, Yamamura A, Shibata C, Sasaki I. Cronkhite-Canada syndrome complicated with huge intramucosal gastric cancer. Gastric Cancer. 2009;12(2):113-7
[6] Yashiro M, Kobayashi H, Kubo N, Nishiguchi Y, Wakasa K, Hirakawa K. Cronkhite-Canada syndrome containing colon cancer and serrated adenoma lesions. Digestion 2004; 69(1):57-62.