World Cholangiocarcinoma Day (CCA)

Dr Grainne O’Kane, Consultant Medical Oncologist, St James’s Hospital. Director, Cancer Clinical Trials Unit, Trinity St James’s Cancer Institute

Cholangiocarcinoma (CCA) is a cancer of the bile ducts occurring inside and outside the liver. The number of  CCA diagnosed each year is increasing dramatically around the world. CCA which occurs within the liver known as ‘intrahepatic CCA’  and is often mistaken for a cancer of unknown primary, however the correct diagnosis is extremely important as management options can be very different. Surgery provides the only chance of cure in CCA and when CCA cannot be removed by surgery, chemotherapy or other local treatments such as specialised radiation may be considered. Many centres have also been evaluating liver transplant in clinical trials in certain types of CCA.

In the past 5 years there have been major advances in how we treat patients with CCA. Firstly, understanding the genetic make-up of these cancers  is crucial as approximately 40% of CCA occurring within the liver will have specific gene alterations  that we can treat with targeted therapies. This means that all patients with this type of cancer should have access to these tests. Secondly, immunotherapy is now considered standard of care in combination with chemotherapy as first treatment in patients whose tumours cannot be removed by surgery. Two studies have demonstrated improvements in the numbers of patients surviving with double the number of patients alive at 5 years when compared with chemotherapy alone.

My particular research interests have been in understanding which types of CCA will do better or worse according to their genetic makeup. In particular I have been interested in looking at young onset CCA and patients who develop CCA when they have primary sclerosing cholangitis. This is a well-documented risk factor for this type of cancer. Knowing who might develop CCA when they have primary sclerosing cholangitis is crucial to picking it up early. However in the majority of patients we do not understand why this cancer occurs and much research is needed given the rise in numbers and the aggressiveness of this cancer.

For more information, please see https://www.globalccaalliance.com/world-cca-day