Hilar cholangiocarcinoma: pathology and tumor biology.
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.
Institute of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
Dong
D
Kuang
Guo-Ping
GP
Wang
D000818:Animals; D001650:Bile Duct Neoplasms / Q000453:epidemiology / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D018281:Cholangiocarcinoma / Q000453:epidemiology / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D015209:Cholangitis, Sclerosing / Q000150:complications; D006801:Humans; D009367:Neoplasm Staging; D009374:Neoplasms, Experimental; D012307:Risk Factors