Clin Endosc.  2013 May;46(3):301-305. 10.5946/ce.2013.46.3.301.

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

Affiliations
  • 1Department of Internal Medicine, Presbyterian Medical Center, Jeonju, Korea. yz9017@daum.net

Abstract

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.

Keyword

Intestinal polyposis; Colonic neoplasms; Serrated adenoma; Helicobacter pylori

MeSH Terms

Adenoma
Alopecia
Asian Continental Ancestry Group
Colon
Colonic Neoplasms
Diarrhea
Epidermis
Foot
Gastrointestinal Tract
Hand
Helicobacter pylori
Humans
Hyperpigmentation
Intestinal Polyposis
Korea
Male
Nails
Polyps
Prednisolone
Republic of Korea
Stomach
Weight Loss
Prednisolone

Figure

  • Fig. 1 Patient appearance findings. (A) Hair pull test showed that more than 10 hairs were pricked out. (B) Black pigmentation and (C) nail dystrophy was observed in both hand.

  • Fig. 2 Endoscopic findings. (A) Several hundred 0.5- to 2.5-cm-sized, strawberry-like polyps with normal mucosa in the gastric antrum. (B) Numerous tiny polyps in the small bowel on capsule endoscopy. (C) A 15-mm-sized, grape-shaped polyp (black arrow) with thousands of polyps in the transverse colon (pathologically confirmed adenocarcinoma in situ). (D) A 10-mm-sized, round-shaped polyp (black arrow) in the ascending colon (pathologically confirmed serrated adenoma).

  • Fig. 3 Pathologic findings of colonoscopic polypectomy. (A) Inflamed lamina propria, proliferated tortuous and cystic dilated glands (H&E stain, ×200), (B) adenocarcinoma in situ with background low grade tubular adenomas (H&E stain, ×200), (C) adenocarcinoma in situ (H&E stain, ×400), and (D) serrated adenoma (H&E stain, ×400) were observed.


Cited by  1 articles

Cronkhite-Canada Syndrome Showing Good Early Response to Steroid Treatment
Woohee Cho, Kwangwoo Nam, Ki Bae Bang, Hyun Deok Shin, Jeong Eun Shin
Korean J Gastroenterol. 2018;71(4):239-243.    doi: 10.4166/kjg.2018.71.4.239.


Reference

1. 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; 61:293–309. PMID: 7109958.
2. Dachman AH, Buck JL, Burke AP, Sobin LH. Cronkhite-Canada syndrome: radiologic features. Gastrointest Radiol. 1989; 14:285–290. PMID: 2680733.
Article
3. Goto A. Cronkhite-Canada syndrome: epidemiological study of 110 cases reported in Japan. Nihon Geka Hokan. 1995; 64:3–14. PMID: 8534187.
4. Hizawa K, Nakamori M, Yao T, Matsumoto T, Iida M. A case of Cronkhite-Canada syndrome with colorectal adenomas: effect of the nonsteroidal anti-inflammatory drug sulindac. Am J Gastroenterol. 2007; 102:1831–1832. PMID: 17686082.
Article
5. Riegert-Johnson DL, Osborn N, Smyrk T, Boardman LA. Cronkhite-Canada syndrome hamartomatous polyps are infiltrated with IgG4 plasma cells. Digestion. 2007; 75:96–97. PMID: 17510553.
Article
6. Chadalavada R, Brown DK, Walker AN, Sedghi S. Cronkhite-Canada syndrome: sustained remission after corticosteroid treatment. Am J Gastroenterol. 2003; 98:1444–1446. PMID: 12818298.
Article
7. Yashiro M, Kobayashi H, Kubo N, Nishiguchi Y, Wakasa K, Hirakawa K. Cronkhite-Canada syndrome containing colon cancer and serrated adenoma lesions. Digestion. 2004; 69:57–62. PMID: 14755154.
Article
8. Kang YW, Park SK, Kim H, Bae OS, Chang ES. A case with some components of Cronkhite-Canada syndrome in a family with Peutz-Jeghers syndrome. Korean J Intern Med. 1988; 3:136–141. PMID: 3154191.
Article
9. Lee SK, Kim MY, Choi SH, et al. One case of Cronkhite-Canada syndrome. Korean J Intern Med. 1992; 42:278–281.
10. Choi MS, Kim YT, Jung HC, et al. A case of Cronkhite-Canada syndrome. Korean J Gastroenterol. 1992; 24:154–159.
11. Park UC, Oh MH, Park EU, Kim SY, Seo JM, Park JG. A case report of Cronkhite Canada syndrome in the entire gastrointestinal tract. J Korean Soc Coloproctol. 1992; 8:173–180.
12. Hong SJ, Kwon SH, Kim HJ, et al. A case of Cronkhite-Canada syndrome. Korean J Med. 1996; 51:825–831.
13. Chung ST, Chung EC, Choi JH, Sung KJ, Moon KC, Koh JK. A case of Cronkhite-Canada syndrome. Korean J Dermatol. 1999; 37:381–385.
14. Kim HJ, Jeen YT, Chun HJ, et al. Two cases of Cronkhite-Canada syndrome with remission. Korean J Gastrointest Endosc. 2000; 21:543–548.
15. Goo YS, Shin HJ, Park JY, et al. A case of Cronkhite-Canada syndrome with a remission to steroid therapy. Korean J Gastrointest Endosc. 2001; 23:113–117.
16. Kim MS, Jung HK, Jung HS, et al. A case of Cronkhite-Canada syndrome showing resolution with Helicobactor pylori eradication and omeprazole. Korean J Gastroenterol. 2006; 47:59–64. PMID: 16434870.
17. Park W, Jeon WK, Lee JE, et al. A case of Cronkhite-Canada syndrome conducted with capsule endoscopy of small intestine. Korean J Gastrointest Endosc. 2010; 40:126–129.
18. Baek JH, Kim TY. Cronkhite-Canada syndrome that developed in a patient taking levothyroxine sodium after total thyroidectomy. Korean J Dermatol. 2011; 49:45–49.
19. Lee HJ, Park SJ, Choi HS, et al. A case of Cronkhite-Canada syndrome presenting with hematochezia. Intest Res. 2011; 9:238–242.
Article
20. Ward E, Wolfsen HC, Ng C. Medical management of Cronkhite-Canada syndrome. South Med J. 2002; 95:272–274. PMID: 11846261.
Article
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