Cholangiocarcinoma

Question 1

What is cholangiocarcinoma?

Answer 1

Cholangiocarcinoma = malignancy arising from the bile duct epithelium

Divided based on location in biliary tree
Intrahepatic
Extrahepatic
Perihilar (most common)
Klatskin’s tumours = involving the bifurcation of the ducts
Distal

> 95% are adenocarcinomas
Most commonly infiltrating nodular/diffusely infiltrating

Question 2

What is the aetiology of cholangiocarcinoma?

Answer 2

Most often there is no identifiable aetiology
Infection and inflammation often associated
Chronic hep B/C cirrhosis
PSC (5-15% progress to cholangiocarcinoma)
Liver flukes
Hepatolithiasis

Question 3

What are the risk factors of cholangiocarcinoma?

Answer 3

Age >50

Inflammatory disorders - (Cholangitis, Choledocholithiasis/cholecystolithiasis, UC, PSC)

Infections - (Liver fluke, Typhoid, Hep B/C, HIV)

Alcoholic liver disease

Question 4

What is the epidemiology of cholangiocarcinoma?

Answer 4

2/3 occur in patients aged 50-70

Incidence varies worldwide (fairly low in UK)

Question 5

What are the symptoms of cholangiocarcinoma?

Answer 5

Painless jaundice (90%)

Clay coloured stools

Bilirubinuria

Weight loss

Abdominal pain

Pruritus

Question 6

What are the signs of cholangiocarcinoma?

Answer 6

Courvoisier’s sign - Palpable painless gallbladder

Hepatomegaly

Question 7

What are appropriate investigations for cholangiocarcinoma?

Answer 7

Bloods
LFTs
Obstructive jaundice
Bilirubin: Elevated
ALP: Elevated
GGT: Elevated
ALT, AST

Clotting screen
PT: Prolonged

Cancer markers
Serum Ca 19-9: Elevated (85%)
Also elevated in pancreatic/gastric malignancy, any obstructive jaundice
Serum CEA
Also elevated in IBD, tumours, severe liver injury
Serum Ca 125

Abdo US
Dilated intrahepatic ducts
Intraheptic: Mass lesion

CT/MRI
ERCP -Filling defect or area of narrowing
Brush cytology/biopsy
MRCP

Diagnostic - PET