Zamani Zarlakhta, Fatima Saira
King Edward Medical University (Mayo Hospital Lahore)
Aga Khan University
The biliary tract comprises of gallbladder and intra and extrahepatic biliary tree. Bile is directed through these ducts to the second part of duodenum at major duodenal papilla. The epithelium of the biliary tract is lined with cells called cholangiocytes. Carcinoma of the biliary tract arises from the malignant transformation of the epithelium of the bile ducts which is made up of these cholangiocytes, and is categorized on the basis of its anatomical location as; 1) Intrahepatic cholangiocarcinoma 2) Extrahepatic cholangiocarcinoma, which includes; perihilar tumor also known as Klatskin tumor (originating from the epithelium of the bile duct at the junction of right and left hepatic ducts with the cystic duct where it forms the common bile duct) and distal cholangiocarcinoma outspreading to encompass the gallbladder, ampulla of Vater and pancreatic biliary ducts. Although both intrahepatic and extrahepatic cholangiocarcinoma arises from the same epithelium of the bile duct, the pathogenesis and clinical outcomes of extrahepatic cholangiocarcinoma differs from that of intrahepatic cholangiocarcinoma on the basis of different anatomical location. Cholangiocarcinoma is considered as one of the rare but most aggressive tumors with a very poor prognosis because mostly, it is advanced and unresectable by the time it is diagnosed. Late presentation of the tumor in many cases has already caused the extensive involvement of the blood vessels and the regional lymph nodes that curative surgical resection becomes a challenge. Although rare, it represents the second most common type of primary liver malignancy following hepatocellular carcinoma.
胆道由胆囊以及肝内和肝外胆管树组成。胆汁通过这些导管引流至十二指肠大乳头处的十二指肠第二部。胆道上皮由称为胆管细胞的细胞排列而成。胆管癌起源于由这些胆管细胞组成的胆管上皮的恶性转化,并根据其解剖位置分为:1)肝内胆管癌;2)肝外胆管癌,其中包括肝门周围肿瘤,也称为克氏壶腹周围癌(起源于左右肝管与胆囊管汇合形成胆总管处的胆管上皮)以及远端胆管癌,其扩散范围包括胆囊、 Vater壶腹和胰胆管。尽管肝内胆管癌和肝外胆管癌均起源于胆管的同一上皮,但由于解剖位置不同,肝外胆管癌的发病机制和临床结局与肝内胆管癌有所不同。胆管癌被认为是罕见但侵袭性最强的肿瘤之一,预后很差,因为大多数情况下,在诊断时它已处于晚期且无法切除。在许多病例中,肿瘤的晚期表现已导致血管和区域淋巴结广泛受累,以至于根治性手术切除成为一项挑战。尽管罕见,但它是继肝细胞癌之后第二常见的原发性肝脏恶性肿瘤。