Bile duct cancer

The bile ducts are the thin tubes that carry bile down from the liver and the gallbladder into the bowel. A tumour growing from their wall is called a cholangiocarcinoma. It is uncommon, but it has one feature worth knowing about: for a long time it gives nothing away, and the first sign, often the only one, is jaundice without pain. That is usually how the disease is found. The sooner someone with yellowed eyes reaches a doctor, the more treatment options remain open.

Where exactly the tumour grows

Almost everything depends on which part of the duct system the tumour has arisen in: the symptoms, the tests and whether surgery is possible.

• inside the liver, where the tumour grows silently and is often picked up by chance on a scan done for something else;
• at the hilum, where the ducts join and leave the liver: the commonest site, and the one that blocks the flow of bile early;
• in the lower part of the duct, next to the pancreas and the duodenum.

Once a duct is blocked, bile no longer reaches the bowel and builds up in the blood. That is where the whole familiar picture comes from: the yellow colour, the itching, dark urine and pale stools.

How it starts

• yellowing of the whites of the eyes and of the skin; on brown and black skin it shows up less well, so look at the whites of the eyes, the gums and the palms;
• itching all over the body, often unbearable and frequently weeks ahead of the yellow colour;
• urine the colour of strong tea and pale, almost white stools;
• weight falling without dieting or effort, appetite gone;
• a dull ache or heaviness under the right ribs, nausea;
• constant tiredness, weakness, a sense of being generally unwell;
• a raised temperature and shivering.

None of these signs means cancer on its own. In an adult, jaundice is far more often caused by a stone, by hepatitis or by a medicine. But the cause has to be sorted out every time, and quickly.

When to see a doctor without delay

Seek emergency care straight away if jaundice comes with a high temperature, shivering and pain under the right ribs, or if there is confusion or a sudden drop in blood pressure. This is how infection in a blocked duct presents, and it develops within hours.

The same urgency applies to vomiting blood, black tarry stools, or severe abdominal pain that will not let go.

See a doctor within days, not in a month's time, if:

• the whites of the eyes or the skin have turned yellow, even without pain and without feeling ill;
• the whole body itches with no rash, particularly alongside dark urine;
• weight is coming off by itself;
• stools have become persistently pale.

What raises the risk

In most people who develop it, no clear cause is ever found, so having none of the factors below guarantees nothing. Even so, the odds are higher with:

• primary sclerosing cholangitis, a chronic scarring inflammation of the ducts that often runs together with ulcerative colitis; it is the weightiest known risk factor, and that is exactly why these patients are monitored regularly;
• cirrhosis of the liver and chronic hepatitis B and C;
• congenital cysts and abnormalities in the shape of the bile ducts;
• stones in the ducts and repeated bouts of inflammation;
• liver flukes, common in South-East Asia, worth remembering if the person lived there for a long time;
• being over sixty-five, smoking, excess weight, diabetes.

How the cause is tracked down

Blood comes first: bilirubin and the liver readings, clotting and markers of inflammation. Then come the images. Ultrasound shows whether the ducts are widened, in other words whether the outflow is blocked. Computed tomography and above all magnetic resonance imaging with a dedicated study of the ducts map out the whole layout and show where the obstruction sits and what surrounds it. The inside can also be reached with an endoscope passed through the duodenum: during that procedure material is taken for analysis and, if needed, a small tube or stent is placed there and then so that bile can flow again. If there is no way in from inside, the duct is drained through the skin.

A piece of tissue is not always taken: sometimes the images are convincing enough, and a needle can get in the way of later surgery. This is not carelessness but a deliberate decision, and it is worth asking about.

The extent is then pinned down with a positron emission scan and sometimes a look inside the abdomen through a small incision. Whether surgery goes ahead depends on the result.

A word about the waiting. Results take weeks to come together, and that is the hardest part of all. A long wait does not mean bad news: the scans and the slides are reviewed by several specialists. It is better not to go alone to the appointment where the findings are explained, and to bring the questions written down, because half of them are forgotten in the room.

How it is treated

The plan is put together by a team: a surgeon, an oncologist, an endoscopy specialist and a radiologist. Bile duct cancer can often be held in check for a long time, but getting rid of it altogether is possible only through surgery and only while the spread is limited.

• Surgery is the only route to cure. The affected stretch of duct is removed, frequently along with a lobe of the liver and the surrounding lymph nodes, and sometimes with part of the pancreas. In selected cases of a tumour at the hilum, a liver transplant is considered.
• Chemotherapy is used after surgery to lower the risk of return, and as the main treatment when the tumour has spread.
• Immunotherapy uses drugs that help the immune system recognise the tumour; these are added to chemotherapy in advanced disease.
• Targeted drugs only work when the tumour carries particular genetic changes, so it is worth asking your doctor whether molecular testing of the tissue has been done.
• Radiotherapy and local treatments aimed at a tumour inside the liver are used less often, in specific situations.

When the tumour cannot be removed

That does not mean there is no treatment. The aim changes: to slow the growth, take away what causes suffering and keep life normal for as long as possible.

• a stent or a drain restores the flow of bile: the jaundice fades, the itching settles, appetite returns and chemotherapy becomes possible;
• itching is treated with medicines of its own and does not have to be endured;
• if fatty food is digested poorly, enzymes are prescribed and weight and vitamins are kept under review;
• pain relief is stepped up until the pain is properly controlled, not until some fixed ceiling;
• people with this tumour have a higher risk of clots, so swelling and pain in one leg, or sudden breathlessness, call for immediate attention.

The palliative care team does not come in during the last days but as soon as there are symptoms getting in the way of living. Involving them early makes people feel better and does not cancel out treatment aimed at the tumour.

What helps you get through it

Do not go to appointments alone: the other person remembers what you miss. Keep a list of your medicines and dates on your phone, because it is useful in any hospital. Do not be shy about asking for something to be explained a second time. Eat smaller amounts more often if your appetite has dropped, and keep moving within your strength: even short walks reduce the weakness. Anxiety and low mood are as expected here as nausea, and respond to help just as well, so tell your doctor about them.

Online consultation

In an online consultation the doctor works through what lies behind the jaundice, the itching or the weight loss, explains which tests are needed first and how urgent they are, translates the reports and results you already have into plain language, and suggests what to ask your treating team and how to manage itching, nausea and loss of appetite between visits.

This material is for information only and does not replace medical advice.