Tannapfel A, Wittekind C
Institut für Pathologie, Universitätsklinikum Leipzig.
Internist (Berl). 2004 Jan;45(1):33-41. doi: 10.1007/s00108-003-1110-6.
Biliary tract cancers are a consequence of a stepwise malignant transformation of the biliary epithelium. Intrahepatic cholangiocarcinomas arises from any portions of the intrahepatic bile duct epithelium: the segmental or area ducts and their finer branches or intrahepatic small bile ducts. Cholangiocarcinoma arising from the hepatic ducts or near their junction are called hilar cholangiocarcinoma or Klatskin tumour, and are considered as extrahepatic lesion. Cancer of extrahepatic bile ducts may also arise in the Ductus cysticus or choledochus as well as in the gall bladder. For intrahepatic cholangiocarcinoma, the UICC-TNM classification system of malignant liver tumors is applied, differing from the UICC-TNM staging system of extrahepatic bile ducts and gall bladder carcinomas. The cause of carcinomas of the bile ducts remains speculative in most cases. However, chronic inflammation due to sclerosing cholangitis, hepatolithiasis or parasites is associated with carcinogenesis. Histopathologically, the vast majority are adenocarcinomas; mesenchymal tumors and primary melanomas are extremely rare. Different genetic alterations are discussed to be of importance.
胆管癌是胆管上皮细胞逐步恶性转化的结果。肝内胆管癌起源于肝内胆管上皮的任何部位：节段性或区域胆管及其更细的分支或肝内小胆管。起源于肝管或其附近交界处的胆管癌称为肝门部胆管癌或克氏瘤，被视为肝外病变。肝外胆管癌也可能发生在胆囊管或胆总管以及胆囊。对于肝内胆管癌，采用恶性肝肿瘤的UICC-TNM分类系统，这与肝外胆管癌和胆囊癌的UICC-TNM分期系统不同。在大多数情况下，胆管癌的病因仍具有推测性。然而，硬化性胆管炎、肝内胆管结石或寄生虫引起的慢性炎症与致癌作用有关。在组织病理学上，绝大多数是腺癌；间叶性肿瘤和原发性黑色素瘤极为罕见。不同的基因改变被认为具有重要意义。
