24th July 2023

Cholangiocarcinoma

Cancer of the bile duct, which may be intrahepatic, extrahepatic, or perihilar (Klatskin tumours), depending on the exact location within the duct in relation to the liver. Histologically, these tumours are adenocarcinomas. Generally advanced at presentation and therefore associated with a poor prognosis – early diagnosis is key. Median survival is around 6 months for those with metastatic disease.

Causes and risk factors

Cholangiocarcinoma are often idiopathic, and there are few identified risk factors. There is some association with chronic parasitic infections (particularly liver flukes), and Asian ethnicity, the latter of which may be due to an increased prevalence of parasitic infections in these countries.

Primary sclerosing cholangitis (see below) has a key causal link, and individuals with this condition should have close surveillance for the development of cholangiocarcinoma.

Clinical features

Signs and symptoms do not typically present until disease is advanced, which is why diagnosis is often late. The characteristic features are jaundice, pruritus, weight loss, anorexia, pale stools, and dark urine. Abdominal pain may be present but is often absent; painless jaundice is, therefore, a major red flag feature, which often distinguishes cholangiocarcinoma from disease relating to gallstones.

Assessment and diagnosis

LFTs will show a cholestatic picture (elevated ALP/GGT/bilirubin) and are often grossly deranged.

Ultrasound is often the first imaging done, as it is non-invasive and easy to arrange, and can rule out other causes. ERCP is the preferred diagnostic investigation, as it can directly demonstrate the tumour and be used to obtain histological samples. CT is also done for diagnostic and staging purposes.

Management

Surgery is the only curative treatment, but many cholangiocarcinomas are inoperable. Radiotherapy and chemotherapy are used both adjuvantly with surgery and palliatively for unresectable disease.