Hilar Cholangiocarcinoma

Overview

Hilar cholangiocarcinoma is a rare type of cancer that starts in the bile ducts, the small tubes that carry a yellow-green fluid called bile away from your liver and into your gut. Bile helps you digest fats. The word 'hilar' means the cancer begins in the area called the hilum, which is the spot just where the bile ducts leave the liver. Doctors sometimes call it a Klatskin tumour. Because the bile ducts are very narrow there, even a small growth can block the flow of bile, which is why jaundice (yellowing of the skin and eyes) is often one of the first things people notice. It is a serious condition, and care is usually arranged through a specialist liver or cancer centre.

No, it is rare. Hilar cholangiocarcinoma affects roughly one to two people per 100,000 each year in many countries, and it makes up a small share of all cancers of the bile ducts. Because it is uncommon, general doctors may see very few cases in their whole career, which is one reason people are usually referred to a specialist centre.

It mostly affects older adults, usually in their 60s and 70s, and it is very rare in children and young adults. It can affect anyone, but it is more likely in people with certain long-term bile duct or liver conditions, and in parts of the world where a parasite infection of the liver is common. Men are slightly more likely to be affected than women in many countries.

Symptoms

Common symptoms

• Yellowing of the skin and the whites of the eyes (jaundice)
• Pale, clay-coloured stools and dark urine
• Itchy skin, which can be widespread and hard to ignore
• Tiredness and a general feeling of being unwell
• Losing weight without trying, or losing your appetite
• Discomfort or aching in the upper right part of the belly, sometimes felt in the back or right shoulder
• Feeling sick or being sick
• A mild fever, or feeling chilled

Symptoms in children

• This cancer is extremely rare in children, so there is little specific information for this age group
• If a child develops jaundice, pale stools or unexplained weight loss, they should be checked by a doctor quickly, as there are many other more common causes to look into first

Symptoms in older adults

• Symptoms may come on quietly and be put down to getting older, such as tiredness, poor appetite or weight loss
• Jaundice may be noticed first by family or friends rather than the person themselves
• Confusion or unusual drowsiness can appear with jaundice and needs urgent medical attention
• Older adults may also have other health conditions, so a full review by a doctor is important before any treatment decisions

Causes

Main causes

• Hilar cholangiocarcinoma happens when cells lining the bile ducts develop changes in their genetic material, called mutations, that make them grow out of control. In most people there is no single cause that can be pointed to. Doctors do not fully understand why it develops in any one person, and it is not caused by anything a person did or did not do.

Risk factors

• Primary sclerosing cholangitis, a long-term condition that causes scarring and inflammation of the bile ducts
• Inflammatory bowel disease, especially ulcerative colitis, which often occurs alongside primary sclerosing cholangitis
• Choledochal cysts, which are abnormal pouches in the bile ducts present from birth
• Long-standing bile duct stones or repeated bile duct infections
• Infection with a liver fluke parasite, which is seen mainly in parts of Southeast Asia and is picked up by eating raw or undercooked freshwater fish
• Cirrhosis, which is scarring of the liver from any cause
• Long-term infection with hepatitis B or hepatitis C
• Smoking, obesity, and diabetes have been linked with a modestly higher risk
• Some rare inherited conditions, including Lynch syndrome and certain bile salt disorders
• Increasing age, and being male, both of which are linked to a higher chance of developing it

When to see a doctor

See a doctor urgently if:

• You notice your skin or the whites of your eyes turning yellow
• Your stools have become pale and your urine very dark
• You have jaundice with fever, chills or shaking
• You have severe pain in the upper belly that is getting worse
• You have lost weight quickly without trying
• You have itching all over that is not settling with simple measures

Book a routine appointment if:

• You feel more tired than usual, or your appetite has changed for several weeks
• You have ongoing discomfort in the upper right part of your belly
• You have long-term primary sclerosing cholangitis or a bile duct condition and want to talk about your check-ups
• You have a family history of bile duct or liver cancer and want advice about your risk

Diagnosis

There is no simple single test for hilar cholangiocarcinoma. Your doctor will start by asking about your symptoms and health history and will examine you, including checking your eyes and skin for jaundice. Blood tests usually come next, followed by scans of your liver and bile ducts. If these suggest a growth in the bile ducts, a specialist team will confirm it by taking a small sample of tissue, called a biopsy, and will also work out how far the cancer extends, which is called staging. This information is used to plan the best possible treatment for you.

Tests that may be done

• Blood tests to check how your liver is working and to look for signs of blockage or infection
• Blood tests for tumour markers, which are substances that can be raised in some cancers; these give clues but cannot confirm a diagnosis on their own
• An ultrasound scan of the tummy, often the first scan done
• A CT scan or MRI scan to look closely at the liver, bile ducts and nearby structures
• A specialised MRI scan that shows the bile ducts clearly, using a dye given into a vein
• An endoscopy test where a thin camera is passed near the bile duct opening so dye can be injected and pictures taken, and a sample can be collected
• A procedure where a fine needle is passed through the skin into the liver or bile ducts to take pictures or a tissue sample
• A biopsy, where a small piece of tissue is examined under a microscope to confirm the type of cancer
• A keyhole operation to look inside the tummy and check whether the cancer has spread, in some cases before planning surgery

What to expect at your appointment

The tests usually happen over a few weeks and can feel like a lot of waiting. Some, such as blood tests and scans, are quick and painless. Others, such as an endoscopy or a biopsy, may need you to stop eating for a few hours beforehand and may be done with sedation so you are comfortable. You may be asked to come to a specialist liver or cancer centre. It helps to bring someone with you, write down your questions, and ask for the results to be explained in plain language. Waiting for results is often the hardest part emotionally, so plan some support around that time.

Treatment

Treatment for hilar cholangiocarcinoma is planned by a team of specialists, including liver surgeons, cancer doctors, radiologists and specialist nurses. The plan depends on where the cancer is, how far it has spread, how well your liver is working, and what matters most to you. There are two main goals: to remove or control the cancer, and to relieve jaundice, itching and blockage so you feel better. Some people are treated with the aim of curing the cancer, while for others the focus is on controlling it and keeping you comfortable for as long as possible. You should be fully involved in these decisions, and it is fine to ask for time to think or to seek a second opinion.

Self-care at home

• Keep to any appointment and scan schedule your team gives you, and report new symptoms early
• Take care of your skin if it is itchy: keep baths lukewarm, pat skin dry, use unperfumed moisturiser, keep nails short, and ask your team about anti-itch options
• Eat small meals more often if your appetite is small, and ask to speak to a dietitian for practical advice
• Sip fluids regularly through the day, especially in hot weather or if you feel sick
• Note down changes in your symptoms, weight or urine and stool colour so you can share them at appointments
• Avoid alcohol while your liver is under strain, and check with your team before taking any new medicine, supplement or herbal product, including ones bought without a prescription
• Ask your team which vaccinations and travel precautions are right for you

Medical treatments

Treatment approaches fall into a few broad groups. Surgery aims to remove the affected bile ducts and part of the liver, and is the only treatment that can offer a cure; it is only suitable for some people. For people who cannot have surgery, doctors may use chemotherapy, which is medicine that attacks cancer cells, sometimes combined with radiotherapy, which uses carefully targeted X-rays. Some people are offered newer treatments aimed at specific features of their cancer cells, or treatments that help the immune system fight the cancer; these depend on tests on the cancer tissue and are not suitable for everyone. Alongside this, an important part of care is relieving the blockage: a small tube called a stent can be placed inside the bile duct to let bile flow again, which reduces jaundice and itching. Supportive and palliative care teams work alongside the cancer team to control pain, sickness, itching and tiredness at any stage of the illness, not only at the end. Your doctors will explain the likely benefits and drawbacks of each option for your situation, and you can change your mind as things change.

When is surgery considered?

Surgery is considered when the cancer is found before it has spread beyond the liver and nearby bile ducts, and when your liver is healthy enough to cope with part of it being removed. It is a major operation done at a specialist centre, and you would have detailed tests first to check whether it is safe and likely to help. Sometimes surgery is not possible because the cancer has grown into blood vessels or has spread, or because the liver is too damaged. If surgery is not an option, the team will focus on other treatments and on keeping you as well as possible. In a small number of carefully selected people, a liver transplant may be considered within a specialist programme.

Living with this condition

Living with this diagnosis usually means a mix of hospital appointments, treatment, and ordinary life. Many people feel very tired, and energy can vary from day to day, so it helps to pace yourself and accept help with chores and shopping. Jaundice and itching can affect how you feel about your appearance and can be uncomfortable, but there are practical things that help. Keep a simple diary of symptoms, medicines and questions, and take it to appointments. Ask your specialist nurse to be your main point of contact, since they can often sort out problems quickly between appointments.

Lifestyle tips

• Rest when your body asks for it, and plan activities for the times of day when you have most energy
• Ask family and friends for specific help, such as lifts to hospital or help with meals
• Keep up gentle movement if you can; short walks can help with tiredness and mood
• Avoid alcohol, and check with your team before taking any new medicine, vitamin or herbal remedy
• Stay in touch with work or studies if you want to, and ask about flexible arrangements
• Let your team know early about fever, new pain, or changes in your stent, as these are easier to treat when caught quickly

Diet and exercise

There is no special diet that treats this cancer, but eating well helps you cope with treatment and keep your strength. If your appetite is poor, try small portions more often, choose foods you enjoy, and keep easy snacks within reach. A dietitian at your hospital can give personalised advice, including about high-calorie drinks and protein. Some people need extra help with vitamins, particularly vitamin D, so ask your team rather than starting supplements yourself. Gentle activity, such as short daily walks, is usually helpful and safe, but build up slowly and stop if you feel unwell. Ask your doctor what level of exercise is right for you, especially around surgery or treatment cycles.

Mental health and emotional wellbeing

Being told you have a rare cancer can bring shock, fear, anger and sadness, and it is normal to feel overwhelmed at times. Waiting for scan results and planning treatment can be especially stressful. Sleep, appetite and concentration often suffer. Talking about how you feel helps, whether with family, a specialist nurse, a counsellor or a support group. If low mood, anxiety or hopelessness is lasting more than a couple of weeks or is affecting daily life, please tell your doctor or nurse, because good help is available. If you ever have thoughts of harming yourself or ending your life, contact your local emergency number or a crisis line in your country straight away, and stay with someone you trust until help arrives.

Prevention

There is no sure way to prevent hilar cholangiocarcinoma, and most people who develop it have no obvious risk factor. However, some steps may lower the chance of liver and bile duct problems in general: not smoking, keeping to a healthy weight, limiting alcohol, and getting treated for hepatitis B or C if you have it. People with primary sclerosing cholangitis or choledochal cysts are usually followed up regularly by a specialist, which is a sensible way to pick up changes early. If you are travelling to areas where liver fluke infection is common, avoid eating raw or undercooked freshwater fish.

Vaccines

There is no vaccine against this cancer. There is a vaccine against hepatitis B, which prevents a long-term liver infection that can lead to liver damage and cancer; it is part of routine childhood immunisation in many countries and is available for adults at higher risk. Ask your doctor whether it is recommended for you.

Screening programmes

There is no general screening programme for hilar cholangiocarcinoma, because it is too rare for population screening to be useful. People with high-risk conditions such as primary sclerosing cholangitis may be offered regular check-ups and scans as part of their specialist care. If you have a condition that raises your risk, ask your specialist how often you should be reviewed.

Complications

If left untreated

• Bile can build up in the liver, causing deepening jaundice, intense itching and damage to liver cells
• Blocked bile ducts can become infected, which can lead to a serious infection that spreads through the body and needs emergency treatment
• The cancer can spread to nearby lymph nodes, blood vessels, or other parts of the body such as the lungs or bones
• Weight loss, poor appetite and tiredness can lead to frailty and a lower ability to cope with treatment
• Liver failure can develop if the liver is badly affected

Long-term outlook

Hilar cholangiocarcinoma is a serious cancer, and it is honest to say that the outlook varies a great deal from person to person. When the cancer is found early and can be removed with surgery, some people live for many years and some are cured. When surgery is not possible, treatment can still control the cancer, relieve symptoms and help people feel better for longer. Everyone's situation is different, and statistics cannot tell you what will happen in your own case. Your specialist team can talk through what is known about your particular cancer and what choices are available to you, and support is there at every stage.

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