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Frontiers of Medicine in China

, Volume 4, Issue 4, pp 371–377 | Cite as

Hilar cholangiocarcinoma: Pathology and tumor biology

  • Dong Kuang
  • Guo-Ping WangEmail author
Review

Abstract

Hilar cholangiocarcinoma, first described by Klatskin in 1965, is a relatively rare tumor arising from the bile ducts. The histomorphological features of hilar cholangiocarcinoma are identical with other extra- and intra-hepatic bile duct carcinomas. The most common disease associated with cholangiocarcinoma is primary sclerosing cholangitis. The development of cholangiocarcinoma is a multistep process associated with several mutations in oncogenes and tumor-suppressor genes. Based on macroscopic appearance, three distinct subtypes have been described: sclerosing, nodular, and papillary. Microscopically, more than 95% of tumors are adenocarcinomas. Hilar cholangiocarcinoma is a slowly growing tumor and tends to spread longitudinally along the bile ducts with neural, perineural, and subepithelial extension. Lymph node invasion can be found in 30%–50% patients at the time of diagnosis, but blood-born metastases are rare and usually occur at late stages.

Keywords

hilar cholangiocarcinoma morphology primary sclerosing cholangitis metastasis growth 

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Copyright information

© Higher Education Press and Springer-Verlag Berlin Heidelberg 2010

Authors and Affiliations

  1. 1.Institute of Pathology, Tongji Hospital, Tongji Medical CollegeHuazhong University of Science and TechnologyWuhanChina

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