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For further information, see CMDT Part 39-07: Carcinoma of the Biliary Tract

Key Features

Essentials of Diagnosis

  • Obstructive jaundice, usually painless, often with dilated biliary tract

  • Pain is more common in gallbladder carcinoma than cholangiocarcinoma

  • A dilated gallbladder may be detected (Courvoisier sign)

  • Diagnosis by cholangiography with biopsy and brushings for cytology

General Considerations

Gallbladder carcinoma

  • Onset is notoriously insidious; diagnosis is often made unexpectedly at surgery

  • Cholelithiasis (often large, symptomatic stones) usually present

  • Risk factors

    • Chronic infection of the gallbladder with Salmonella typhi

    • Adenomatous gallbladder polyps > 1 cm in diameter (particularly with hypoechoic foci on endoscopic ultrasonography [EUS])

    • Mucosal calcification of the gallbladder (porcelain gallbladder)

    • Anomalous pancreaticobiliary ductal junction

    • High parity in women

    • Increased body mass index

    • Aflatoxin exposure

    • Genetic factors include K-ras and TP53 mutations

  • TNM staging for gallbladder carcinoma

    • Tis, carcinoma in situ

    • T1a, tumor invades lamina propria

    • T1b, tumor invades muscle layer

    • T2, tumor invades perimuscular connective tissue with no extension beyond serosa (visceral peritoneum) (T2a) or into liver (T2b)

    • T3, tumor perforates the serosa or directly invades the liver or adjacent organ or structure

    • T4, tumor invades the main portal vein or hepatic artery or invades two or more extrahepatic organs or structures

    • N1, metastasis to 1–3 regional lymph nodes

    • N2, metastasis to ≥ 4 regional lymph nodes

    • M1, distant metastasis

Carcinoma of the bile ducts (cholangiocarcinoma)

  • About 50% arise at the confluence of the hepatic ducts (perihilar or so-called Klatskin tumors), and 40% arise in the distal extrahepatic bile duct (the incidence of which has risen since 1990); the remainder are intrahepatic (the incidence of which rose dramatically from the 1970s to early 2000s and has continued to increase)

  • Risk factors for intrahepatic cholangiocarcinoma

    • Hepatitis C virus infection (and possibly hepatitis B virus infection)

    • Cirrhosis

    • HIV infection

    • Nonalcoholic fatty liver disease

    • Diabetes mellitus

    • Obesity

    • Tobacco smoking

  • Mixed hepatocellular carcinoma-cholangiocarcinoma is being increasingly recognized

  • In southeast Asia, hepatolithiasis, chronic typhoid carriage, and infection of the bile ducts with helminths (Clonorchis sinensis, Opisthorchis viverrini) are associated with an increased risk of cholangiocarcinoma

  • TNM staging for perihilar cholangiocarcinoma

    • Tis, carcinoma in situ/high-grade dysplasia

    • T1, tumor confined to bile duct

    • T2, tumor invades beyond the wall of the bile duct to surrounding adipose tissue (2a) or to the adjacent liver (2b)

    • T3, tumor invades unilateral branches of the portal vein or hepatic artery

    • T4, tumor invades the main portal vein or its branches bilaterally, common hepatic artery, second-order biliary radicals, and contralateral portal vein or hepatic artery

    • N1, metastasis to 1–3 regional lymph nodes

    • N2, metastasis to ≥ 4 regional lymph nodes

    • M1, distant metastasis

  • TNM staging for intrahepatic cholangiocarcinoma

    • T1a, solitary tumor ≤ 5 cm without vascular invasion

    • T1b, solitary tumor > 5 cm without vascular invasion

    • T2, solitary tumor with intrahepatic vascular invasion or multiple tumors ≤ 5 ...

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