Thousands of young and seemingly healthy Britons are at risk of a once-rare form of aggressive liver cancer because GPs are unaware of how to detect it, a new campaign has warned.
In the UK, cases of bile duct cancer, which affects tubes in the liver, have doubled in the past two decades according to AMMF, the UK’s only charity for the cancer – and scientists still don’t know what is behind the surge.
However, research suggests the deadly disease, once thought to only affect older people, is now striking a growing number of patients below the age of 50. And, unlike other forms of liver disease, bile duct cancer does not seem to be triggered by lifestyle habits – such as excessive drinking, smoking or obesity.
Nor does the cancer – also known as cholangiocarcinoma – appear to run in families, in contrast to common cancers such as breast and prostate.
This means many new cases arise seemingly at random, making it difficult to catch early.
Yet despite the dangers, experts claim many GPs do not know what the telltale symptoms of the disease are, so patients are regularly diagnosed too late to be cured.
Disturbingly, NHS cancer diagnosis guidelines designed to help family doctors spot signs of the disease include no mention of cholangiocarcinoma – even though the symptoms are different to other liver cancers.
There are also serious concerns over the competency of NHS cancer doctors assigned to treat bile duct cancer patients. Only six experts in the disease are believed to be employed by the entire NHS, and insiders have told The Mail on Sunday that many cancer specialists are unaware of how to effectively treat it.
Mail on Sunday journalist Katie Nicholl, 47, who was diagnosed with bile duct cancer in 2023 after suffering indigestion and heart palpitations
Shockingly, research suggests that around half of patients diagnosed with cholangiocarcinoma are simply given painkillers because doctors wrongly believe nothing can be done to save them.
Campaigners are now calling on the Government to approve regulations that would see it included in the list of cancers GPs are required to look for.
One of those supporting the change is Mail on Sunday journalist Katie Nicholl, 47, who was diagnosed with bile duct cancer in 2023 after suffering indigestion and heart palpitations.
At first, the Royal correspondent thought fatigue or the perimenopause could be to blame for these mild symptoms.
She had spent the previous 12 months writing two books, and has covered the death of Queen Elizabeth and the Coronation of King Charles for US TV network NBC and the BBC. But as Katie revealed in yesterday’s Daily Mail, it was only after a series of fortunate interventions that her cancer was spotted.
First, a friend urged Katie to seek medical help. Her GP put the symptoms down to stress but referred her to a cardiologist.
After scans and blood tests failed to show any signs of disease, the heart specialist agreed to send Katie for one final investigation. That scan – an ultrasound of her abdomen – revealed a lesion growing on her liver. And, despite assurances from doctor that it was unlikely to be cancer due to her age, further tests confirmed the presence of cholangiocarcinoma.
Katie with her children Matilda and George. She made the decision not to tell her children she had cancer and instead told them she needed to go to hospital for an operation on her liver
Katie with Dr Dora Pissanou, one of the country’s leading liver surgeons, who performed the 11-hour procedure to cut open Katie’s stomach to remove the tumour
Ten days later, at the Royal Free Hospital in north London, Katie was sedated and her stomach was cut open for surgeons to remove the tumour. The 11-hour procedure was performed by Dr Dora Pissanou, one of the country’s leading liver surgeons.
Katie spent 12 days recovering in hospital. Mercifully, follow-up scans revealed she was cancer-free. Katie continues to get these scans every three months.
But she worries that many other cholangiocarcinoma patients are slipping through the net due to a lack of knowledge about the disease among GPs.
‘Everyone, myself included, first thought my symptoms were down to stress,’ she told The Mail on Sunday. ‘And it was only because I pushed for scans that my cancer was detected early enough for there to be a very good prognosis for me.
‘I’ve always been healthy. I eat well, exercise regularly, don’t smoke and I’m not a big drinker. But when I started getting heart palpitations, I had this gut feeling that something wasn’t right.
‘I was lucky to have a GP who listened to me. After my surgery I wrote to thank him for referring me to a specialist. He called and told me that I was the first case of bile duct cancer he’d ever seen, and that it wasn’t a condition on the radar of GPs.
‘He said he’s since put my card in the staff room as a reminder to the other doctors to keep an eye out for this horrible disease.’
She added: ‘It’s so easy to miss, and I realise I’m hugely lucky to still be alive. We need to do everything we can to make sure GPs know the symptoms of cholangiocarcinoma. Many patients aren’t as lucky as I am.’
Two years after getting the all-clear, Katie says she feels better – and fitter – than ever. She plans to run the Hackney Half Marathon in May in aid of the charity Maggie’s which provides free expert care and support for cancer patients.
So what is cholangiocarcinoma – and what are the symptoms?
The disease affects the tubes in the liver that carry bile, a fluid that helps the body digest food by breaking down fat.
Cases have been on the rise in the UK over the past 20 years, and campaign group AMMF says it has been hearing from an increasing number of young patients, some only in their 20s. In 2023, The Mail on Sunday revealed the number of cholangiocarcinoma patients equalled all other forms of liver cancer.
Hepatocellular carcinoma – previously the most common form of liver cancer – usually occurs as a result of liver cirrhosis. This scarring of the liver is often caused by alcohol abuse or obesity.
Ilona Smith, 44, from south London was diagnosed with cholangiocarcinoma in 2020 after she began to experience severe back and liver pain
Fashion designer Zandra Rhodes – who has created outfits for Princess Diana and Elizabeth Taylor, among others – was diagnosed with cholangiocarcinoma in 2020 at the age of 80
NHS patients diagnosed with liver cirrhosis are offered a scan every six months to look for any potentially cancerous changes.
But the majority of cholangiocarcinoma patients do not have liver cirrhosis, and cases are typically picked up only when the disease is advanced, causing fatigue, abdominal pain, weight loss, itchiness and jaundice.
Some 6,000 people die from liver cancer in the UK every year. But experts say they are still unsure what triggers cholangiocarcinoma.
‘Many who come to us after their diagnosis do not fit the bill of a typical cancer patient,’ says AMMF chief executive Helen Morement. ‘These are often healthy people who don’t smoke, don’t drink and rarely eat red meat.’
Not all cholangiocarcinoma patients are young. Fashion designer Zandra Rhodes – who has created outfits for Princess Diana and Elizabeth Taylor, among others – was diagnosed with it in 2020 at the age of 80.
Experts have raised the possibility the rise in cases could be linked to ultra-processed foods (UPFs) – those created with artificial ingredients such as preservatives and flavourings.
Common UPFs include ready meals, cereals and mass-produced bread. And research suggests they account for nearly 60 per cent of calories consumed by the average UK adult.
‘The problem is that it’s unbelievably difficult to prove this,’ says Professor John Bridgewater, a gastrointestinal oncologist at University College London Hospitals NHS Foundation Trust. ‘All we know is that cholangiocarcinoma is on the rise, but there is very little awareness of it, not just among the public but within the healthcare profession too.’
Experts say any patient with the suspected signs of cholangiocarcinoma should receive a liver function blood test, to see if it is working properly. GPs can order this, which is cheap and easy to carry out.
‘If there is anything abnormal then they can be referred on to hospital for scans, which will find the cancer if it’s there,’ says Prof Bridgewater.
However, experts say the early symptoms are often mistaken for non-serious conditions. One of the most common complaints that patients report is indigestion – thought to be due to tumours blocking the bile duct, meaning the fluid can’t get to the stomach.
‘We often hear from patients who were first told they probably had IBS [irritable bowel syndrome] and get sent home with some tablets,’ says Ms Morement. ‘The number of patients with the classic cholangiocarcinoma symptoms who are essentially flicked away by GPs is shocking.’
Experts say this situation is made worse by the fact that NHS cancer guidance issued to GPs includes no mention of cholangiocarcinoma. But hepatocellular carcinoma, the other main type of liver cancer, is mentioned.
One crucial difference in symptoms is that cholangiocarcinoma can trigger pale stools and dark-coloured urine (often likened to stewed tea), while hepatocellular carcinoma does not.
But there is no information available to GPs warning them that these symptoms could be a sign of liver cancer.
Experts say another major issue is that – even after patients are diagnosed – many cancer specialists do not know how to treat cholangiocarcinoma.
Only a limited number of patients are able to undergo surgery to remove the bile duct tumours. This is because, often, by the time the tumours are found they have grown too big to be safely removed without risking life-threatening damage to the liver itself. Moreover, surgery is not an option for patients whose cholangiocarcinoma has spread outside the liver.
Until recently, this has meant that chemotherapy was the only option for many patients. But it usually cannot cure the cancer, which is why so many patients are given nothing but painkillers.
In recent years, a number of game-changing immune-boosting drugs have become available in the UK, which can extend the lives of patients by years and, in some cases, shrink their tumours enough to make surgery possible.
Research suggests that about half of all cholangiocarcinoma patients could benefit from these new treatments.
‘These targeted treatments can be life-changing for patients,’ says Prof Bridgewater. ‘They are three or four times more effective than chemo.
‘And we’re even finding that some patients see their tumours shrink so much that their cancer goes from being inoperable to operable, opening up the possibility of curing them.’
But in order to access these immunotherapies, a piece of the tumour has to be removed and sent for testing, known as a molecular profiling. The immunotherapy that patients receive will depend on the genetic make-up of the tumour.
‘All my patients get molecular profiling, but I worry about those living elsewhere in the country, in more rural areas for example, who are missing out,’ says Prof Bridgewater.
One patient who benefited from molecular profiling is Ilona Smith, 44, from south London. She was diagnosed with cholangiocarcinoma in 2020 after she began to experience severe back and liver pain.
Iiona underwent three months of chemo and then surgery to remove the tumour, followed by more chemo. She was given the all-clear but, in May 2022, a scan revealed that it had returned.
Chemo did not work this time, and neither did two experimental drugs. At this point, she was told there were no more treatments.
Then, in May 2023, Ilona was offered molecular profiling. The results showed that there was a drug which was thought to be effective against her cancer. The drug was not available in the UK yet, but Ilona was enrolled in a clinical trial.
She began taking two tablets every day, which had little-to-no side effects and stopped the cancer from growing.
‘I’m still taking the tablets today and they still appear to be working,’ she says. ‘I got lucky because I got molecular profiling, but many people do not.’
To donate to Katie’s fundraiser for Maggie’s cancer charity go to: justgiving.com/page/katie-nicholl-1
Dame Zandra leads fight against disease
Fashion legend Dame Zandra Rhodes and the celebrity photographer Rankin have joined forces with bile duct charity AMMF to raise awareness of the disease.
Ms Rhodes, 84, who created outfits for Princess Diana, was diagnosed with cholangiocarcinoma in March 2020 after noticing swelling in her stomach.
She was initially given six months to live but was put on an immune-boosting drug which proved effective. Four years later, she is still in remission. She features in a series of images shot by Rankin – acclaimed for his pictures of the late Queen, Kate Moss and Madonna – alongside other cholangiocarcinoma patients.
They include The Mail on Sunday’s Katie Nicholl, 47, who reveals her ordeal above.
Other patients featured are Andy Clay, 53, Andrea Sheardown, 53, Judith Neptial, 52, Benjamin Carey, 55 Ilona Smith, 44, and Charlie Shrager, 50.
The Mail on Sunday’s Katie Nicholl, Dame Zandra Rhodes, Andy Clay, Andrea Sheardown, Judith Neptial, Benjamin Carey, Ilona Smith, and Charlie Shrager featured in celebrity photographer Rankin’s photo
