Understanding Cholangiocarcinoma: A Rare Cancer – World Cancer Day 2025
Understanding Cholangiocarcinoma: A Rare Cancer – World Cancer Day 2025
Dr Esra Hassan – Research Fellow in Cancer
4th Feb 2025
It is World Cancer Day—a global
initiative to raise awareness, improve education, and drive action on cancer. But
this day also falls within Cholangiocarcinoma Awareness Month, a time
dedicated to increasing recognition of bile duct cancer, one of the rarest and
most aggressive cancers. Despite its severity, it remains under-recognised,
often diagnosed too late for curative treatment.
Let’s take a moment to understand
cholangiocarcinoma, recognise its symptoms, and explore why early detection and
research are critical. Awareness can save lives.
What is Cholangiocarcinoma?
Cholangiocarcinoma is a malignant
tumour originating in the bile ducts, the small tubes that carry bile from the
liver and gallbladder to the intestines. It accounts for approximately 3% of
all gastrointestinal cancers and has an incidence rate of 1-2 cases per 100,000
people annually. In 2019, cholangiocarcinoma was the cause of 2,754 deaths in
England alone.
While bile duct cancer, is considered rare, worldwide it is the second most common primary liver cancer. It is difficult to diagnose accurately and early, so by the time of diagnosis it is often too advanced for surgery – which is currently the only potential cure. Although most cases occur in people over 60 years of age, this disease appears to be increasing across all age groups including younger people. The cause of this ongoing rise is currently unknown.
The cancer is categorised by its location into three main types based on where it starts:
✅ Intrahepatic – inside the liver
(accounts for 10-20% of cases)
✅ Perihilar (hilar) – where the
bile ducts exit the liver (accounts for 50-60% of cases)
✅ Distal – near the pancreas and small intestine (accounts for 20-30% of cases)
Stages of Cholangiocarcinoma
Most cases are diagnosed at later
stages because symptoms like jaundice, weight loss, and abdominal pain often
appear only when the cancer has progressed.
Cholangiocarcinoma is staged
using the TNM staging system (Tumour, Node, Metastasis). Early-stage cancers
(Stages I and II) are localised, while advanced stages (III and IV) involve
lymph node spread or distant metastases. Unfortunately, more than 70% of cases
are diagnosed at Stage III or IV, significantly limiting treatment options.
Why Does Early Detection Matter?
Like many cancers, early
diagnosis means better survival. But cholangiocarcinoma is often diagnosed at
an advanced stage, when surgery—the only potential cure—is no longer possible.
📊 If caught early (Stage
1), up to 30% of patients survive 5 years after surgery.
📊 For advanced stages,
survival is often less than 1 year.
Who is at Risk?
Risk factors for
cholangiocarcinoma include Chronic bile duct inflammation (e.g., primary
sclerosing cholangitis), have certain medical conditions, such as abnormal bile
ducts, and ulcerative colitis. However, many patients present with no known
risk factors, making universal screening unfeasible. Instead, improving
awareness of symptoms could help patients seek care sooner.
Symptoms to Look Out For
Early symptoms can be vague, but
being aware of them could help people seek medical advice sooner. If you or
someone you know experiences these symptoms persistently, it’s worth seeing a
doctor.
Most people don’t experience symptoms until the cancer is quite advanced, but here are key warning signs:
⚠️ Jaundice – Yellowing of the
skin and eyes (a sign that bile flow is blocked).
⚠️ Unexplained weight loss –
Losing weight without trying.
⚠️ Abdominal pain – Especially in
the upper right side.
⚠️ Dark urine and pale stools –
Due to bile duct blockage.
⚠️ Itchy skin – Caused by bile
build-up in the bloodstream.
Tests for bile duct cancer
🩸 Blood tests – These
check liver function and look for signs of bile duct blockage or tumour
markers.
🖥 Scans – Imaging tests
like ultrasound, CT scans, or MRI scans help doctors visualise the bile ducts
and surrounding organs. Sometimes, an endoscopic ultrasound (EUS) is used,
where a small camera is passed into the body via the digestive tract.
🧪 Biopsy – A small sample
of cells from the bile ducts is collected and examined under a microscope to
check for cancer. This can be done during an ERCP or through a needle biopsy
guided by imaging scans.
📡Endoscopic Retrograde
Cholangio-Pancreatography (ERCP) – This specialised test uses an endoscope (a
thin, flexible tube with a camera) to examine the bile ducts.
📷 Percutaneous
Transhepatic Cholangiography (PTC) – A special type of X-ray where a fine
needle is inserted through the skin into the liver to inject contrast dye,
making the bile ducts visible on X-ray images. This helps detect narrowing or
blockages caused by cancer.
A combination of these tests is
usually needed to confirm a diagnosis and determine the best treatment
approach.
Current Treatment Landscape
Treatment options for
cholangiocarcinoma depend on the stage of diagnosis:
💊 Surgery – Surgical
resection offers the best chance of a cure but is possible for fewer than 30%
of patients, as most are diagnosed too late. Procedures include Liver resection
for intrahepatic tumours and Whipple procedure for distal tumours.
💊 Chemotherapy – For
advanced or unresectable cancers, standard treatment for advanced cases to help
slow progression is chemotherapy. Gemcitabine and cisplatin are the standard
first-line treatments, offering a median survival of 11.7 months.
💊 Targeted therapies –
New drugs designed for tumours with specific genetic mutations (e.g., FGFR2
inhibitors). Drugs include pemigatinib and infigratinib for tumours with specific
genetic mutations.
💊 Immunotherapy – An
emerging area of research showing promise. Trials of checkpoint inhibitors like
pembrolizumab have shown promise.
💊Photodynamic therapy
(PDT): This uses light-activated drugs to kill cancer cells and relieve
symptoms.
Advances in Research and Early Detection
Research is moving toward
personalised medicine, where treatments are tailored to the genetic and
molecular profile of a patient’s tumour. For example, testing for mutations in
IDH1, BRAF, or FGFR2 helps identify candidates for targeted therapies. However,
we are not there yet. Early detection remains the biggest challenge. Blood
biomarkers like CA 19-9 and CEA are commonly used but lack specificity. Novel
biomarkers and imaging techniques, like contrast-enhanced MRIs, are being
developed to improve diagnostic accuracy.
🚀 More research funding
is needed to improve early detection and treatment.
🩺 Greater awareness among
both doctors and the public can lead to faster diagnosis.
❤️ Better support for patients
and families facing this rare cancer is crucial.
Raising awareness is the first step toward change. Here’s how
you can help:
✅ Share the symptoms – Someone
might recognise symptoms earlier.
✅ Encourage screening – People
with risk factors (e.g., liver disease) should speak to their GP.
✅ Advocate for policy changes: Push for more funding into early detection and innovative treatments.
#WorldCancerDay #BileDuctCancer
#CholangiocarcinomaAwareness #CholangiocarcinomaAwarenessMonth - Written with the assistance of AI
In memory of Helena West
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