Cholangiocarcinoma
- Lesion
Cholangiocarcinoma is cancer of the bile duct lining; it grows slowly along the ducts, causes jaundice and is often found late.
What it is
Cholangiocarcinoma is an adenocarcinoma of biliary epithelium. By site it is intrahepatic, perihilar (at the confluence of the hepatic ducts, the Klatskin tumour) or distal. Chronic biliary inflammation and stasis are the shared background: primary sclerosing cholangitis, liver flukes, hepatolithiasis, choledochal cysts and Caroli disease. Precursors include biliary intraepithelial neoplasia, intraductal papillary neoplasms and mucinous cystic neoplasms. The tumour is desmoplastic and spreads along nerves and ducts.
Structure
- Gross: grey-white firm mass (intrahepatic), or a stricture with thickened duct wall (perihilar and distal), sometimes papillary.
- Micro: glands of cuboidal or columnar cells, often mucin-producing, in sclerotic stroma; lymphatic and perineural invasion.
Step by step
- Chronic inflammation or stasis (PSC, flukes, stones, cysts) injures biliary epithelium.
- Cytokines such as IL-6 and repeated regeneration favour mutations.
- Epithelium becomes dysplastic: biliary intraepithelial neoplasia, then high grade.
- Invasive glands provoke dense desmoplastic stroma and narrow the duct.
- The tumour spreads along ducts, nerves and lymphatics; obstruction causes jaundice and cholangitis.
Cholangiocarcinoma by location
| Type | Location | Typical presentation | Molecular clues |
|---|---|---|---|
| Intrahepatic | Ducts within the liver, proximal to second-order ducts | Liver mass, often without jaundice | IDH1/IDH2 mutations, FGFR2 fusions |
| Perihilar (Klatskin) | At or near the confluence of the right and left hepatic ducts | Painless obstructive jaundice | KRAS, TP53 |
| Distal | Common bile duct down to the ampulla | Painless jaundice, palpable gallbladder | KRAS, TP53, SMAD4 |
Markers
- Keratin 7
- Keratin 19
- CA 19-9 (serum)
- Albumin in-situ hybridization (intrahepatic)
Test yourself
Think of the answer, then tap the question to see it.
What is a Klatskin tumour?
A perihilar cholangiocarcinoma arising at the confluence of the right and left hepatic ducts.
Which parasitic infections predispose to cholangiocarcinoma?
Liver flukes of the Clonorchis and Opisthorchis groups.
Which targetable alterations are typical of intrahepatic cholangiocarcinoma?
IDH1 or IDH2 mutations and FGFR2 gene fusions.
Connections
Microorganisms1
Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.
Sources: What Is Bile Duct Cancer (Cholangiocarcinoma)? - NCI (National Cancer Institute, 2026).
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Representative model, for educational purposes only.