Treatment options

Cholangiocarcinoma is serious illness and is relatively rare.  It is essential that patients are cared for by knowledgeable specialists so that they are properly assessed and offered the right treatment and management for their particular situation.

For information on requesting a referral for a further opinion to ensure a diagnosis is correct, and for reassurance that the treatments suggested are the best options available, click here


Molecular Profiling and Targeted Therapies

No two tumours are exactly the same. The genetic characteristics of a cancer will vary from one patient to the next, which means that even patients with the same type of cancer may respond differently to the same treatment.

Current scientific advances mean that cancer treatments are moving towards those more specifically targeting the tumour ‘drivers’, and ways to inhibit these drivers. Progress is beginning to be made in the understanding of cholangiocarcinoma, with studies being carried out to find target agents, and also genomic or molecular profiling, in the search for better and more effective treatments.

Update 22 July 2021: NICE has now approved pemigatinib (Pemazyre®) for those with cholangiocarcinoma who have an FGFR2 fusion.  Pemigatinib is the first ever targeted therapy approved for CCA.  This approval will not only give those eligible patients in England and Wales access to an alternative to chemotherapy, but importantly this new NICE guidance now means molecular profiling will be mandated as standard of care and so opens the door for all those diagnosed with cholangiocarcinoma to have molecular testing carried out.  

For AMMF’s report on the NICE approval for pemigatinib, click here.
For the statement from NICE, click here

For a short animated film explaining, “Cholangiocarcinoma and the importance of molecular testing”, click here

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Treatment of cholangiocarcinoma will depend on the position and size of the cancer and whether it has spread beyond the bile duct, as well as on general health. The main options are:


Surgery

Surgery to completely remove the cancer is currently the only potentially curative treatment for cholangiocarcinoma. This involves a major operation and, often, because the disease is too far advanced, or the patient is already too poorly, surgery is not possible. The decision about whether an operation to remove the cancer can be done depends on the results of the diagnostic tests, and on the patient’s general health.

If surgery is possible, the patient must be referred to a surgeon who specialises in biliary tract cancer surgery. The type of operation that is done depends on the size of the cancer and whether it has begun to spread into nearby tissues.

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Stent Insertion

One of the frequent symptoms of cholangiocarcinoma is jaundice.  This happens because the bile duct or ducts become blocked, preventing the normal flow of bile from the liver to the intestines.   To allow the bile to flow again, a stent (usually a metal stent) will be inserted into the bile duct to hold it open.  This will relieve the symptoms of jaundice, the patient’s general health will then improve and they are able to digest food again normally.   (If chemotherapy has been recommended, it is essential this happens before it can begin.)

A stent usually needs to be replaced every 3-4 months as they can become blocked, and cause a biliary tract infection (cholangitis), which can rapidly become serious.  Symptoms of a blocked stent/infection include:

  • high temperature/fever
  • jaundice
  • chills, shivering

If these symptoms develop, it is important to contact the doctor or CNS (Clinical Nurse Specialist) for advice, as antibiotic treatment may be needed and the stent may need to be replaced.

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Chemotherapy

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. There are now a number of chemotherapies used for those with cholangiocarcinoma.

The most commonly used chemotherapy regimes used for those with cholangiocarcinoma are listed below, and for each of these there is downloadable patient information describing the treatment, how it is given, for how long, what side effects might occur and how to deal with them.  

Gemcitabine and Cisplatin (Gem/Cis)
First line standard of care chemotherapy treatment for those with inoperable cholangiocarcinoma is usually a combination of Gemcitabine (Gemzar®) and Cisplatin.

This standard of care chemotherapy combination was adopted worldwide following the results of the UK’s ABC-02 trial in 2010.  Although a number of clinical trials have been carried out since that time, this has not yet been improved on.

For a Patient Information Sheet on the Gemcitabine/Cisplatin chemotherapy, click here

Capecitabine
The chemotherapy capecitabine is now offered to patients following surgical resection for their cholangiocarcinoma.

The results of the long running BILCAP showed that this improved survival.  For more information see: https://ammf.org.uk/2017/05/18/bilcap-results-show-chemo-improves-survival.

For a Patient Information Sheet on Capecitabine, click here

FOLFOX
A second line treatment, the combination of folinic acid, fluorouracil and oxaliplatin (FOLFOX) may be offered to those patients with advanced or metastatic cholangiocarcinoma who have already received first line treatment with the combination chemotherapy Gemcitabine/Cisplatin.

For a Patient Information Sheet on FOLFOX, click here

Other chemotherapies your oncologist may advise for you include:

CAPOX (Capecitabine and oxaliplatin):
For a Patient Information Sheet on CAPOX, click here

Gemcitabine (given alone):
For a Patient Information Sheet on Gemcitabine, click here

Clinical trials continue to investigate ways to better, more effective treatments. For more information on current trials, click here

For further information on the diagnosis and treatment of cholangiocarcinoma, including chemotherapies, see “Guidelines for Diagnosis and Treatment UK” and also,  “International Guidelines”


Radiotherapy

Radiotherapy, which uses high energy x-rays to destroy cancer cells, is not routinely used to treat cholangiocarcinoma, although there may be occasions when it is used palliatively (to reduce symptoms).


SBRT (Stereotactic body radiotherapy)
SBRT gives radiotherapy from several different positions around the body, with the beams meeting at the tumour.  In this way, the tumour receives a high dose of radiation, but the tissues around it receive only a low dose, which lowers the risk of side effects.  SBRT is only suitable for those whose cancer has not spread to other parts of the body.  

SBRT may be given by a LINAC machine (a medical linear accelerator).  These machines are also known by the brand name of the manufacturer, such as CyberKnife and TrueBeam.

A clinical trial, ABC-07, using a combination of SBRT and Gemcitabine/Cisplatin chemotherapy for eligible biliary tract cancer patients is now open.  For more information, click here


SIRT
Please note:  NHS England funding for SIRT, which had been available for patients with intrahepatic cholangiocarcinoma through the CtE (Commissioning through Evaluation) programme ceased on 31st March, 2017.

However, for those meeting the eligibility criteria, SIRT is still available privately, or by making an application for its use under exceptional circumstances.

SIRT (Selective Internal Radiation Therapy, also known as radio-embolisation) is a special type of internal radiotherapy that targets liver tumours inside the body with high doses of radiation.

SIRT involves injecting millions of tiny radioactive ‘beads’ called microspheres into the main blood vessel of the liver through a long thin tube (catheter). The microspheres travel through the blood to the liver and lodge themselves in the very small blood vessels in and around the liver tumours, where they give off high doses of radiation. As the microspheres only give off radiation to a small area, they target the liver tumour while doing little damage to the surrounding healthy liver tissue. The action of the radiation destroys the liver tumour cells causing the tumours to shrink.

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Referral Information 

For information on requesting a referral for a further opinion to ensure a diagnosis is correct, and for reassurance that the treatments suggested are the best options available, click here

July 2021