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Rare Diseases Q1 2021

A ticking time bomb? The rare liver cancer on the rise in younger people

iStock / Getty Images Plus / Ridofranz

Helen Morement

Founder and CEO, AMMF

Cholangiocarcinoma (CCA), or bile duct cancer, is a liver cancer occurring within the biliary system. It is the world’s second most common primary liver cancer.

The number of CCA cases per year, and annual death rates, are rising globally. AMMF’s data study, presented at the EASL 2020 Congress (European Association for the Study of the Liver) shows that in 2017, CCA was the cause of death of 2,523 people in England alone1 – a higher mortality than cervical cancer (8502).

Currently considered a rare cancer, if the year-on-year increase in incidence continues, within a very few years CCA will be over the rare cancer threshold of 6:100,000.

Although current data shows that most cases of CCA occur in people over 60 years of age, there is a worrying increase in younger people. Research to find causes, better tests, more effective treatments and ultimately a cure, has never been more important.

Symptoms of Cholangiocarcinoma

CCA causes few symptoms in its early stages. In more advanced disease, symptoms may include: –

  • Weight loss
  • Yellowing of the skin and eyes (jaundice)
  • Itchy skin
  • White stools
  • Fatigue
  • Abdominal pain

Possible causes

The cause is likely to be a combination of factors, including other illnesses that cause chronic damage to the liver and/or bile ducts, certain toxins and possibly a small genetic predisposition, although it is not thought to be a directly inherited disease.

For the majority of people diagnosed with CCA however, there appears to be no specific or known cause.

Treatment options

  • Surgery – Surgery to completely remove the cancer is currently the only potentially curative treatment. Unfortunately, many patients are too far advanced for this.
  • Stent Insertion – One of the symptoms of CCA is jaundice, when the bile duct or ducts become blocked by the cancer, preventing the normal flow of bile. A small hollow tube – a stent – will be inserted into the bile duct to hold it open, allow the bile to flow again and so relieve the symptoms of jaundice.
  • Chemotherapy – Those with inoperable CCA will be offered the standard of care chemotherapy.
  • Radiotherapy – Not routinely used for CCA, but a current trial, looking at a very precise type of radiotherapy, SBRT (Stereotactic Body Radiotherapy), is currently under way.
  • Clinical Trials – there are currently several clinical trials, including those investigating targeted therapies, open to people diagnosed with CCA.

For more information visit: www.ammf.org.uk

Registered charity no 1091915

1Taken from AMMF’s own data project, presented at the EASL 2020 Congress (European Association for the Study of the Liver) and due to be published later this year:
“Cholangiocarcinoma in England – a national study examining changes in incidence, diagnostic routes and overall survival”
EASL 2020, Poster THU-508. (Poster attached for reference) https://easl.eu/wp-content/uploads/2020/07/Digital-ILC-Scientific-programme-2020-Post-COVID-19.pdf

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