The medical term for bile duct cancer is ‘cholangiocarcinoma’, or ‘CCA’. Cholangiocarcinoma is a cancer that affects the bile ducts, which are tiny tubes that drain bile out of the liver to the intestines. 

The overall risk (incidence) of CCA over the lifetime of an individual with PSC is 10-15%. However, most occur within the first 1-2 years of PSC diagnosis. After those two years, CCA is very rare for people with PSC; less than 1% per year develop the cancer after that time. 

A liver transplant is an operation to remove your liver and bile ducts, and replace it with a healthy one from a donor. There is excellent information about liver transplantation on the NHSBT website (the body that oversees transplantation in the UK): https://www.nhsbt.nhs.uk/organ-transplantation/liver/. If you need to have a transplant, your transplant centre will give you support and detailed information.

There are seven liver transplant centres in the UK (Birmingham, Leeds, Edinburgh, Cambridge, Newcastle and two in London). Your transplant will take place in one of these centres; usually the one nearest to you.

Everyone with PSC and CCA must be cared for by knowledgeable specialists so that they are properly assessed and offered the right treatment and management for their particular situation. 

Liver transplantation has been used to help a small proportion of people with PSC and CCA in the USA and a few other countries.  It is now available to some (not all) people with CCA in the UK, depending on the size and nature of the cancer.

There are three types of CCA, and only some can be treated with liver transplantation:

1. Intrahepatic CCA (iCCA) originates in the bile ducts inside the liver
2. Hilar or perihilar CCA (pCCA) originates just outside the liver, where the left and right hepatic ducts join together
3. Extrahepatic or distal CCA (dCCA) originates in the common bile duct

Liver transplantation is part of the treatment available for some people with PSC and iCCA or pCCA.

pCCA (perihilar CCA)

Only some people with PSC and pCCA will be suitable for a liver transplant. Your doctor will discuss this with a team of experts before proposing transplant as a possible option. We expect 8-10 people a year will be suitable.

When someone with PSC has a liver transplant for pCCA in the UK, they also have a new type of radiotherapy called PBT (Proton Beam Therapy) and chemotherapy before the transplant. 

PBT is a very precise form of radiotherapy that only delivers the radiation in the tumour, avoiding the healthy parts of the liver around it. PBT is thought to improve the chances of a successful transplant and to keep the tumour under control while waiting for the transplant.

There are two state-of-the art centres in the UK that offer PBT (Manchester and London). The PBT will last for three weeks and accommodation will be offered to those who do not live close by.

Having a liver transplant for pCCA in the UK is very new and will only be available as part of a research study called EMPHATIC. This is so that evidence can be built up about how effective it is.

iCCA (Intrahepatic CCA)

Only some people with iCCA will be suitable for a liver transplant. Your doctor will discuss this with a team of experts before proposing transplant as a possible option. We expect 6-8 people a year will be suitable.

To be eligible for a liver transplant, those with iCCA must have background chronic (long-term) liver disease and not be suitable for a resection (surgery), and the tumour should be less than 3cm in size.

If your doctor thinks transplant might be an option for you, your suitability will be assessed at one of the seven UK liver transplant centres.

When you are listed for a liver transplant, you are added to a national waiting list until a suitable liver is found for you. Because it can take many months (and sometimes years) to get a liver transplant, transplants for CCA are expected to go ahead within 3 months of being listed for transplant. If you are waiting for longer than 3 months, you will be assessed again to make sure you are still suitable.

1. For more information on CCA, go to AMMF.
2. For more information about PSC go to PSC Support - so you need a liver transplant?.