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

• The diagnosis is often made unexpectedly at surgery

• Cholelithiasis (often large, symptomatic stones) is usually present

• Risk factors

  • Chronic infection of the gallbladder with Salmonella typhi

  • Gallbladder polyps over 1 cm in diameter (particularly with hypoechoic foci on endoscopic ultrasonography [EUS])

  • Mucosal calcification of the gallbladder (porcelain gallbladder)

  • Anomalous pancreaticobiliary ductal junction

  • 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 one to three regional lymph nodes

  • N2, metastasis to four or more 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 or peripheral (the incidence of which rose dramatically from the 1970s to early 2000s)

• 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

  • T3, tumor invades to liver, gallbladder, or pancreas or a single vein or artery in the liver

  • 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 one to three regional lymph nodes

  • N2, metastasis to four or more regional lymph nodes

  • M1, distant metastasis

• TNM staging for intrahepatic cholangiocarcinoma

  • T1a, solitary tumor ≤ 5 cm

  • T1b, solitary tumor > 5 cm without vascular invasion

  • T2, solitary tumor with intrahepatic vascular invasion or multiple tumors ≤ 5 cm with or without vascular invasion

  • T3, tumor perforating the visceral peritoneum

  • T4, tumor invading adjacent organ (except gallbladder)

  • N1, regional lymph node metastasis

  • M1, distant metastasis

• Risk factors for intrahepatic cholangiocarcinoma

  • Hepatitis C 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 and infection of the bile ducts with helminths (Clonorchis sinensis, Opisthorchis viverrini) is ...