Cholangiocarcinoma

Question 1

What is a cholangiocarcinoma (CCA).

Answer 1

It is an uncommon tumour that can arise anywhere in the biliary tree, from the intrahepatic bile ducts (20-25% of cases) and the confluence of the right and left hepatic ducts at the liver hilum (50-60%) to the distal common bile duct (20%).

Question 2

What proportion of primary liver cancers are CCAs.

Answer 2

10%.

Question 3

What are the causes of CCA. (10)

Answer 3

Flukes (Clonorchis). 
PSC. 
Gallstones. 
Biliary cysts. 
Caroli's disease. 
HBV. 
HCV. 
DM.
Biliary enteric drainage surgery. 
N-nitroso toxins.

Question 4

What are the symptoms of CCA. (6)

Answer 4

Fever. 
Abdominal pain. 
Possibly ascites. 
Malaise. 
Presentation is typically obstructive jaundice. 
Patients may lose weight.

Question 5

What is raised on the blood tests of a patient with CCA. (2)

Answer 5

Raised bilirubin.

Raised alkaline phosphate.

Question 6

What are the pathological characteristics of CCAs. (2)

Answer 6

Slow growing.

Most are distal extrahepatic or perihilar.

Question 7

What percentage of all malignancies are due to CCA.

Answer 7

1.5%.

Question 8

What is the lifetime risk of developing CCA in a patient with PSC.

Answer 8

20%.

Question 9

What is the pattern of metastasis for CCAs. (2)

Answer 9

Tumours typically invade the lymphatics and adjacent vessels, with a predilection for spread within perineural sheaths.

Question 10

How is the diagnosis for CCA usually made.

Answer 10

Combination of CT and MRI.

Question 11

What difficulty is there in diagnosing CCA.

Answer 11

It is difficult to confirm diagnosis via imaging in patients with PSC.