Biliary Tract Carcinoma

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 ...