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Semin Liver Dis 2006; 26(1): 042-051
DOI: 10.1055/s-2006-933562
Copyright © 2006 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Primary Sclerosing Cholangitis and Cholangiocarcinoma

Konstantinos N. Lazaridis1 , Gregory J. Gores1
  • 1Division of Gastroenterology and Hepatology, Center for Basic Research in Digestive Diseases, Mayo Clinic College of Medicine, Rochester, Minnesota
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Publication History

Publication Date:
23 February 2006 (online)

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ABSTRACT

Primary sclerosing cholangitis (PSC), a cholestatic liver disease characterized by fibrosing inflammatory damage of the biliary tree, is a risk factor for cholangiocarcinoma (CCA). Indeed, the prevalence of CCA in patients with PSC ranges from 7 to 13%. The major challenges of CCA in PSC patients relate to lack of methods for early diagnosis and the absence of effective treatment. Early diagnosis of CCA in PSC is delayed because its clinical presentation can mimic benign dominant biliary strictures. Moreover, biliary and serum tests to diagnose development of CCA in PSC are limiting, although the use of advanced cytologic techniques for aneuploidy and chromosomal aberrations are promising in this regard. As a result, current therapies do not extend survival with the exception of protocol liver transplantation available to a selected group of patients. Future studies should emphasize deciphering the sequence of events that transform the inflammatory changes of the biliary tree to cancer. Only then will chemoprevention, early diagnosis, and therapy of CCA in patients with PSC improve.

KEYWORDS

Bile duct cancer - inflammation - malignant transformation - primary sclerosing cholangitis

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Gregory J GoresM.D. 

Center for Basic Research in Digestive Diseases, Mayo Clinic College of Medicine

200 First Street SW, Rochester, MN 55905

Email: gores.gregory@mayo.edu