Clinical Endoscopy

Cronkhite-Canada Syndrome Associated with Serrated Adenoma and Malignant Polyp: A Case Report and a Literature Review of 13 Cronkhite-Canada Syndrome Cases in Korea: So Hee Yun, Jin Woong Cho, Ji Woong Kim, Joong Keun Kim, Moon Sik Park, Na Eun Lee, Jae Un Lee, Young Jae Lee; Clin Endosc 2013;46(3):301-305. Published online May 31, 2013; DOI: https://doi.org/10.5946/ce.2013.46.3.301

Cronkhite-Canada syndrome (CCS) is a rare nonfamilial polyposis syndrome characterized by epithelial disturbances both in the gastrointestinal tract and in the epidermis. The pathologic finding of the polyp is usually a hamartomatous polyp of the juvenile type; however, the possibility of serrated adenoma associated malignant neoplasm was reported in some Japanese cases. Up till now in South Korea, 13 CCS cases have been reported, but there was no case accompanied by the colon cancer. We report the first case of CCS associated with malignant colon polyp and serrated adenoma in Korea. A 72-year-old male patient who complained of diarrhea and weight loss was presented with both hands and feet nail dystrophy, hyperpigmentation, and alopecia. Endoscopic examination showed numerous hamartomatous polyps from the stomach to the colon. The pathologic results confirmed colon cancer and serrated adenoma. Helicobacter pylori eradication and prednisolone was used. Thus, the authors report this case along with a literature review.

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Autosomal Dominant Inherited Cowden's Disease in a Family: Jun-Wook Ha; Clin Endosc 2013;46(1):85-90. Published online January 31, 2013; DOI: https://doi.org/10.5946/ce.2013.46.1.85

Abstract PDF PubReader ePub

Cowden's disease, also known as a kind of phosphatase and tensin homolog (PTEN) hamartoma tumor syndrome, is an uncommon autosomal dominant inherited complex disorder with various hamartomatous growths of multiple organs involving all three germ cell layers. It usually manifests with polyps throughout the gastrointestinal tract, ranging anywhere from 30% to 85%, and more common extra intestinal findings. Mucocutaneous lesions like facial trichilemmomas, acral keratoses, papillomatous papules and macrocephaly, and malignancies including breast, thyroid and endometrial carcinoma are the hallmark of the disease. Here we report on familial Cowden's diseases case of a 52-year-old male proband with mucocutaneous lesions and mutation on the PTEN gene obtained by extrapolating from gastrointestinal polyposis as a starter and his daughter who developed thyroid cancer.

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스테로이드 치료에 관해를 보인 Cronkhite - Canada 증후군 1 예 ( A Case of Cronkhite - Canada Syndrome with a Remission to Steroid Therapy ): Korean J Gastrointest Endosc 2001;23(2):113-117. Published online November 30, 2000

Abstract PDF: Cronkhite-Canada syndrome is characterized by generalized gastrointestinal polyposis, ectodermal changes, and the eventual development of diarrhea and weight loss. The pathogenesis of the disease is unknown, and there is no established therapy. The disease has a poor prognosis because of malnutrition resulting from altered absorption in the gastrointestinal tract. We experienced a case of a 56-year-old female with Cronkhite-Canada syndrome. After enteral nutrition and administration of prednisolone for 3 months, clinical improvement was noted with cessation of diarrhea, increased serum protein, disappearance of pigmentation, and regrowth of the scalp hair, finger- and toenails, Endoscopy showed resolution of the gastrointestinal polyposis. So we report here a case of diffuse gastrointestinal polyposis which has been in remission with steroid therapy, (Korean J Gastrointest Endosc 2001;23:113-117)

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관해를 보인 Cronkhite - Canada 증후군 2예 ( Two Cases of Cronkhite - Canada Syndrome with Remission ): Korean J Gastrointest Endosc 2000;21(1):543-548. Published online November 30, 1999

Abstract PDF: The Cronkhite-Canada Syndrome (CCS) is a monfamilial disorder of adults characterized by diffuse gastro-intestinal polyposis, ectodermal changes consisting of alopecia, dystrophy of nails and cutaneous hyper-pigmentation. The pathogenesis and the causes of CCS remain unknwon but the symptoms such as diarrhea and malnutrition are generally progressive, and the prognosis is knwon to be poor. Though the definitive treatment is not well known, it has been reported that the conservative management is the most important treatment, and that the clinical sourse can be reversible. One case was experienced involving Cronkhite-Canada Syndromen in a patient who was managed by conservative treatment and an other who was treated by corticosteroid. These two cases are reported herein with a review of corresponding literature. (Korean J Gastrointest Endosc 2000; 21:543-548)

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