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Cronkhite-Canada syndrome - A Case report.
Anticancer Res. 2016 Aug; 36(8):4215-7.AR

Abstract

BACKGROUND/AIM

Cronkhite-Canada syndrome (CCS) is a non-inherited, sporadic disorder characterized by generalized gastrointestinal polyps (hamartomas), cutaneous pigmentation, alopecia and onychodystrophy. More than 500 CCS patients have been reported, mostly from Asian countries. Patients with CCS have a propensity to develop colonic traditional serrated adenomas (TSA). Some authors found increased frequency of colonic carcinomas in CCS patients. In the present communication, we report a patient with CCS in whom a colonoscopic examination failed to disclose a coexistent TSA.

CASE REPORT

A 73-year-old female had a history of alopecia and nail atrophy. Because of iron deficiency anemia and occult gastrointestinal bleeding, she underwent a colonoscopic examination.

RESULTS

Colonoscopy revealed multiple broad-based polyps. Due to continuous bleedings, a coloproctectomy was performed four months after colonoscopy. Pathology disclosed 50 hamartomas and, unexpectedly, a TSA with high-grade dysplasia in the cecum.

CONCLUSION

The TSA was either overlooked at colonoscopy or not interpreted as different from the other colonic polyps by the endoscopist. CCS cases are very rare in Western countries. Given this circumstance, it is suggested that, when confronting the next CCS case, endoscopists should perform a comprehensive colonoscopic examination, including chromoscopy and directed biopsies from irregular polyps, to rule out a TSA, an adenoma prone to evolve into invasive carcinoma.

Authors+Show Affiliations

Department of Pathology, Center for Digestive Diseases Karolinska University Hospital, Stockholm, Sweden Carlos.Rubio@ki.se.Department of Medicine, Center for Digestive Diseases Karolinska University Hospital, Stockholm, Sweden.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

27466534

Citation

Rubio, Carlos A., and Jan Björk. "Cronkhite-Canada Syndrome - a Case Report." Anticancer Research, vol. 36, no. 8, 2016, pp. 4215-7.
Rubio CA, Björk J. Cronkhite-Canada syndrome - A Case report. Anticancer Res. 2016;36(8):4215-7.
Rubio, C. A., & Björk, J. (2016). Cronkhite-Canada syndrome - A Case report. Anticancer Research, 36(8), 4215-7.
Rubio CA, Björk J. Cronkhite-Canada Syndrome - a Case Report. Anticancer Res. 2016;36(8):4215-7. PubMed PMID: 27466534.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cronkhite-Canada syndrome - A Case report. AU - Rubio,Carlos A, AU - Björk,Jan, PY - 2016/05/24/received PY - 2016/06/14/accepted PY - 2016/7/29/entrez PY - 2016/7/29/pubmed PY - 2017/1/26/medline KW - Cronkhite-Canada syndrome KW - endoscopy KW - traditional serrated adenoma SP - 4215 EP - 7 JF - Anticancer research JO - Anticancer Res. VL - 36 IS - 8 N2 - BACKGROUND/AIM: Cronkhite-Canada syndrome (CCS) is a non-inherited, sporadic disorder characterized by generalized gastrointestinal polyps (hamartomas), cutaneous pigmentation, alopecia and onychodystrophy. More than 500 CCS patients have been reported, mostly from Asian countries. Patients with CCS have a propensity to develop colonic traditional serrated adenomas (TSA). Some authors found increased frequency of colonic carcinomas in CCS patients. In the present communication, we report a patient with CCS in whom a colonoscopic examination failed to disclose a coexistent TSA. CASE REPORT: A 73-year-old female had a history of alopecia and nail atrophy. Because of iron deficiency anemia and occult gastrointestinal bleeding, she underwent a colonoscopic examination. RESULTS: Colonoscopy revealed multiple broad-based polyps. Due to continuous bleedings, a coloproctectomy was performed four months after colonoscopy. Pathology disclosed 50 hamartomas and, unexpectedly, a TSA with high-grade dysplasia in the cecum. CONCLUSION: The TSA was either overlooked at colonoscopy or not interpreted as different from the other colonic polyps by the endoscopist. CCS cases are very rare in Western countries. Given this circumstance, it is suggested that, when confronting the next CCS case, endoscopists should perform a comprehensive colonoscopic examination, including chromoscopy and directed biopsies from irregular polyps, to rule out a TSA, an adenoma prone to evolve into invasive carcinoma. SN - 1791-7530 UR - https://www.unboundmedicine.com/medline/citation/27466534/Cronkhite_Canada_syndrome___A_Case_report_ L2 - http://ar.iiarjournals.org/cgi/pmidlookup?view=long&pmid=27466534 DB - PRIME DP - Unbound Medicine ER -
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