【摘 要】
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Hilar cholangiocarcinoma, first described by Klatskin in 1965, is a relatively rare tumor arising from the bile ducts. The histomorphological features of hilar
【机 构】
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Institute of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and
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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-bom metastases are rare and usually occur at late stages.
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