Abstract
Cronkhite–Canada Syndrome (CCS) is a rare non-inherited gastrointestinal polyposis syndrome with characteristic ectodermal changes. We report an extremely unusual case of CCS associated with primary esophageal and gastric cancers. A 74-year-old Japanese man with symptoms of anorexia and diarrhea was found to have primary esophageal and gastric cancers, as well as multiple gastric and colonic polyps. Based on the physical findings of onychodystrophy and alopecia, we diagnosed CCS. Because of his age and nutritional status, we decided to perform total gastrectomy for gastric cancer and chemoradiotherapy for esophageal cancer, upon completion of which the patient was started on steroid therapy for the CCS. After 1 week of steroid therapy, the patient’s watery diarrhea improved. We recommend that for patients with CCS, the therapeutic strategy be carefully considered based on the patient’s nutritional status, the severity of the CCS, and the extent of gastrointestinal cancer.
References
Cronkhite LW Jr, Canada WJ. Generalized gastrointestinal polyposis; an unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia. N Engl J Med. 1955;252:1011–5.
Ward EM, Wolfsen HC. Review article: the non-inherited gastrointestinal polyposis syndromes. Aliment Pharmacol Ther. 2002;15:333–42.
Goto A, Shimokawa K. Cronkhite–Canada syndrome associated with lesions predisposing to development of carcinoma. J Jpn Soc Cancer Ther. 1986;41:1597–608.
Kaneko Y, Kato K, Tachimori Y, Watanabe H, Ushio K, Yamaguchi H, et al. Triple carcinoma in Cronkhite–Canada syndrome. Jpn J Clin Oncol. 1991;21:194–202.
Murata I, Yoshikawa I, Endo M, Tai M, Toyoda G, Abe S, et al. Cronkhite–Canada syndrome: report of two cases. J Gastroenterol. 2000;35:706–11.
Egawa T, Kubota T, Otani Y, Kurihara N, Abe S, Kimata M, et al. Surgically treated Cronkhite–Canada syndrome associated with gastric cancer. Gastric Cancer. 2000;3:156–60.
Yamaguchi K, Ogata Y, Akagi Y, Sasatomi T, Ozaki K, Ohkita A. Cronkhite–Canada syndrome associated with advanced rectal cancer treated by a subtotal colectomy: report of a case. Surg Today. 2001;31:521–6.
Sagara K, Fujiyama S, Kamuro Y, Tashiro A, Sato T. Cronkhite–Canada syndrome associated with gastric cancer. J Gastroenterol. 1983;18:260–6.
Watanabe T, Kudo M, Shirane H, Tomita S, Orino A, Todo A, et al. Cronkhite–Canada syndrome associated with triple gastric cancers: a case report. Gastrointest Endosc. 1999;50:688–91.
Murai N, Fukuzaki T, Nakamura T, Hayashida H, Okazaki M, Fujimoto K. Cronkhite–Canada syndrome associated with colon cancer: report of a case. Surg Today. 1993;23:825–9.
Karasawa H, Miura K, Ishida K, Sase T, Kobayashi T, Kinouchi M, et al. Cronkhite–Canada syndrome complicated with huge intramucosal gastric cancer. Gastric Cancer. 2009;12:113–7.
Watari J, Morita T, Sakurai J, Yamasaki T, Okugawa T, Toyoshima F, et al. Endoscopically treated Cronkhite–Canada syndrome associated with minute intramucosal gastric cancer: an analysis of molecular pathology. Dig Endosc. 2001;23:319–23.
Tanaka Y, Kono S, Hirabayashi T, Hoya Y, Okamoto T, Yanaga K. A case of Cronkhite–Canada syndrome associated with gastric cancer. J Jpn Surg Assoc. 2012;73:847–51.
Yokoyama T, Matsumoto H, Sakamoto N, Ko S, Yamada Y, Nakajima Y. A case of Cronkhite–Canada syndrome with alpha-fetoprotein producing gastric cancer presented with appendicitis. J Jpn Surg Assoc. 2003;64:3052–7.
Konishi T, Yano H, Negishi S, Watanabe H, Motoyoshi M, Watanabe T, et al. Cronkhite–Canada syndrome associated with gastric cancer. J Jpn Surg Assoc. 1998;59:2801–7.
Uchida Y, Kisu T, Mori H, Yamaoka K, Harada S, Mizoguchi T, et al. Cronkhite–Canada syndrome associated with huge intramucosal gastric cancer. Stom Intest. 1986;21:559–63.
Hushida S, Suga T, Akimoto R. A case report of Cronkhite–Canada syndrome associated with hypercarcinoembryonic antigenemia. J Jpn Surg Assoc. 1994;55:2854–8.
Nakamura Y, Takagi S, Omoto R, Makino T, Yoshii T. A case of Cronkhite–Canada syndrome associated with cancer development of a gastric polyp. Stom Intest. 1979;14:1217–22.
Nonomura A, Ohta G, Ibata T, Shinozaki K, Nishino T. Cronkhite–Canada syndrome associated with sigmoid cancer case report and review of 54 cases with the syndrome. Acta Pathol Jpn. 1980;30:825–45.
Yokoyama S, Yamashita H, Moriuchi A, Mochizuki Y, Nakamura Y, Watanabe H, et al. Cronkhite–Canada syndrome associated with adenosquamous carcinoma in gastric polyps. Stom Intest. 1983;18:981–5.
Matsunou H, Konishi F, Hori H, Ikeda T, Sasaki K, Hirose Y, et al. Characteristics of Epstein–Barr virus-associated gastric carcinoma with lymphoid stroma in Japan. Cancer. 1996;77:1998–2004.
Conflict of interest
We have no conflicts of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Ito, M., Matsumoto, S., Takayama, T. et al. Cronkhite–Canada syndrome associated with esophageal and gastric cancers: report of a case. Surg Today 45, 777–782 (2015). https://doi.org/10.1007/s00595-014-0977-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00595-014-0977-2