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This booklet is designed to answer some of the questions that you, or those who care for you, may have about cholangiocarcinoma. It is not meant to replace the discussion between you and your medical team but aims to help you understand more about what is discussed. We are all individuals and cope in different ways and need different lengths of time to adjust to a cancer diagnosis. Your medical team is here to support you. You may experience a wide range of emotions and may find it difficult to remember everything you have been told. You may want to discuss your worries or concerns with your clinical nurse specialist/key worker who can provide help and advice.

Cholangiocarcinoma is another name for bile duct cancer and cancer of the gallbladder, which is a type of liver cancer. Cancers of the bile duct and gallbladder are rare in the UK. However, it is estimated that there are approximately 1,500 people diagnosed with bile duct cancer in the UK each year. Because cholangiocarcinoma is rare, it is treated in specialist liver cancer centres. Below is a diagram showing the position of the bile ducts and gallbladder in the human body. The bile ducts are drainage tubes connecting the liver and gallbladder to the bowel (gut). Bile is a fluid made by the liver and stored in the gallbladder. Its main function is to break down the fats in food during digestion in the bowel. The bile ducts and gallbladder are known as the biliary system. Part of this system is within the liver and the other part is outside the liver.

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The cause of most bile duct cancers is unknown. However, there are a number of risk factors that we think can increase the risk of developing bile duct cancer:

Inflammatory Bowel Disease (IBD) People who have a chronic inflammatory bowel condition known as ulcerative colitis are at higher risk of developing cholangiocarcinoma.

Abnormal bile ducts People who are born with rare (congenital) abnormalities of the bile ducts, such as cysts, are more at risk of developing cholangiocarcinoma.

Infection In Africa and Asia, infection with a parasite known as the liver fluke, as a child, is thought to increase the risk of bile duct cancers in later life.

Chronic liver disease Chronic liver disease either in the form of viral hepatitis (B or C), alcoholic liver disease, or cirrhosis from other causes also increases the risk.

(bowel)

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The most common symptom of cholangiocarcinoma is jaundice. When the cancer develops in the bile ducts it blocks the flow of bile from the liver to the bowel. When this happens the bile flows back into the blood and body tissues and leads to the skin and the whites of the eyes becoming yellow. This is known as jaundice. The urine may also become a dark yellow colour and stools may become pale. Other problems that may occur include skin irritation (causing you to itch), discomfort in the abdomen, loss of appetite, weight loss and episodes of high temperatures (which often suggests an infection).

At the hospital, the doctor will examine you and take blood samples to check your general health and whether your liver is working properly. We may also use the following tests to help confirm the diagnosis:

Ultrasound This is where sound waves are used to make up a picture of the bile ducts and surrounding organs. This will check for any abnormalities, especially bile duct blockage.

CT (Computerised Tomography) scan This will give more detailed pictures of the bile ducts and the surrounding areas by taking a series of x-rays. These are fed into a computer to build up a three dimensional picture of the inside of your body. Most people who have a CT scan are given a drink or an injection to highlight particular areas, allowing them to be seen more clearly.

MRI (Magnetic Resonance Imaging) This is very similar to a CT scan, but uses magnetic fields instead of x-rays. During the scan you will move very slowly through a metal cylinder. The cylinder is a very powerful magnet, so before you go in to the room you will need to inform the staff if you have any metal inside your body (cardiac monitor, pacemaker, surgical clips or bone pins).The test can take about 30 minutes and is pain free.

ERCP (Endoscopic Retrograde Cholangio-pancreatography) This is a special test carried out under sedation to help you relax. The doctor will carefully pass an endoscope (a long, thin flexible telescope with a light on the end of it) through your mouth, into your stomach and small bowel. Looking down the endoscope, the doctor can find the opening through which the bile duct and the duct of the pancreas drain into the small bowel. A dye (known as the contrast medium) is then injected into the ducts, and the doctor will be able to see with x-rays whether there are any abnormalities or any blockage in the duct. If there is a blockage, the doctor may be able to insert a small tube, known as a stent, to allow the bile ducts to open again.

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PTC (Percutaneous Transhepatic Cholangiography) This is a procedure that allows the doctor to get an x-ray picture of your bile duct as an alternative to ERCP. A local anaesthetic will be administered to an area on the right side of your abdomen, and a thin needle will be passed in to your bile ducts in your liver through your skin. A dye will be injected through the needle into the bile duct within the liver. An x-ray will then be taken to see if there is any abnormality or blockage of the ducts.

Angiography This is a procedure that may be carried out if major surgery is being planned. As the bile duct is very close to large blood vessels, we have to sometimes check whether the blood vessels are affected by the tumour. A fine tube is inserted into an artery in your groin and a dye is injected through the tube. The dye circulates in the arteries to make them show up on the x-ray. The angiogram is carried out in a room within the x-ray department.

Biopsy A special needle is used to take a small sample of tissue or cells from the affected area for examination in the laboratory to confirm the diagnosis. Biopsies can be taken through the skin under local anaesthetic, sometimes using ultrasound or CT as guidance, or during ERCP. Biopsy samples can take up to 10 days to process and may need to be repeated if the results are unclear.

EUS (Endoscopic Ultrasound Scan) This scan is similar to an ERCP, but involves a small ultrasound probe being passed down the endoscope to take an ultrasound scan of the pancreas and surrounding structures.

Sometimes one test, rather than giving an answer, can show you need more and different tests. This may feel difficult to cope with, but it is an important part of the process of diagnosis. Specialist liver centres are best at doing these tests. Doctors need to piece together information from all the tests, along side that from your medical history and physical examination, before they can make a diagnosis.

The stage of a cancer is a term used to describe its size and whether it has spread beyond its original site. Knowing this helps doctors to decide on the most appropriate treatment.

Stage 1 (A or B) The cancer is contained within the bile duct or wall, but has not spread locally.

Stage 2 (A or B) The cancer has spread locally into the nearby tissues such as liver, pancreas, small bowel, gallbladder or the nearest lymph nodes.

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Stage 3 (A or B) The cancer is affecting the main blood vessels that take blood to and from the liver, or has spread into the small or large bowel, the stomach or the abdominal wall muscles. Lymph nodes may also be affected.

Stage 4 The cancer has spread to parts of the body well away from the liver, such as the lungs.

There are a number of treatments available and we will discuss the options with you. The type of treatment that you are given will depend on a number of factors, including your general health and other illnesses, the position and size of the cancer in the bile duct and whether the cancer has spread. Cholangioacaricimona is often incurable, unless the entire tumour can be fully surgically removed. If you have been told that a cure is not possible, the treatment offered will be to try and control your cancer for a period of time. It can be very distressing to hear that there is no cure for your cancer, but there are treatments that may still help you. You may find it difficult to decide whether to go ahead with your treatment. If you cannot make a decision about treatment when it is first explained to you, you can always ask for more time to decide. You can, if you wish, choose not to have the treatment or any treatments. We will be able to explain what may happen if you do not have any treatment. It may be that you have concerns about the treatment, so let us know and we will be able to advise you. The treatment options offered to you will have been carefully considered by a team of specialists, including surgeons, oncologists, gastroenterologists, radiologists and pathologists who form the multidisciplinary team (MDT). There is a separate information leaflet about the role of the MDT.

Surgery may be carried out to remove the cancer, if it has not spread. It is not always possible to carry out surgery, as the bile duct is in a difficult position and it may be impossible to remove the cancer completely. There are different operations depending on how big this cancer is and whether it has begun to spread into nearby tissues.

Removal of the bile ducts or partial liver resection If the cancer is small and contained within the ducts, then just the bile ducts containing the cancer are removed. The remaining ducts in the liver are joined to the small bowel, allowing the bile to flow again. If the cancer has begun to spread to the liver, the affected part of the liver is removed along with the bile ducts.

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Whipples operation If the cancer is larger and has spread into nearby structures, then the bile ducts, part of the stomach, part of the small bowel, the pancreas, gallbladder and the surrounding lymph nodes are all removed.

Bypass or palliative Surgery Sometimes it is not possible to remove the tumour and other procedures may be performed to relieve the blockage and allow the bile to go into the bowel. The jaundice will then usually clear up.

Chemotherapy Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy the cancer cells. They work by disrupting the growth of cancer cells. Chemotherapy may also be given in combination with radiotherapy for cancers that cannot be removed surgically or sometimes offered after surgery to prevent the cancer coming back. Chemotherapy may prolong life, but rarely cures this type of cancer on its own. Chemotherapy drugs are usually given by injection into a vein (intravenously) or through a small portable pump, where a controlled amount of chemotherapy can be given continuously into the blood stream over a period of time. Some chemotherapy drugs are now available as tablets, which you can administer yourself. Chemotherapy is almost always given in the outpatient department, which means that you will not have to be admitted to hospital. We will need to take a sample of your blood for testing before each chemotherapy treatment, and it may take a few hours before the doctor receives these results and assesses you for your treatment. If your blood results are satisfactory, we will contact the pharmacy department and ask them to prepare your chemotherapy. The chemotherapy treatment dose is worked out especially for the individual person. This means that it cannot be prepared until we have the results of your blood tests. It can seem like a long day and so you may wish to bring some snacks along with you in case you feel hungry. You can eat and drink normally before, during and after your chemotherapy. Sometimes your hospital doctor will want to delay your chemotherapy for a week or longer. This is because your body needs to recover before the next dose. This is a normal and important part of chemotherapy treatment and does not usually change the overall success of the treatment.

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Specific chemotherapy drugs and information sheets will be given to you. The chemotherapy drugs mostly used are: Gemcitabine, Cisplatin, Oxaliplatin, Fluorouracil (5FU) and capecitabine. New treatments in clinical trials may also be available. Your cancer specialist (oncologist) will be able to discuss the benefits and risks with you, and provide you with further specific information. The aim is to shrink and control the tumour to try to extend life and control symptoms. Chemotherapy given to relieve symptoms is called palliative chemotherapy.

Radiotherapy This treatment is used less often than others to control bile duct cancer. Radiotherapy treats cancer by using high energy x-rays to destroy cancer cells, while doing as little harm as possible to normal cells. It may be given externally from a radiotherapy machine or, rarely, internally by placing radioactive material close to the tumour (this is called brachytherapy). External radiation is given in doses much higher than in standard diagnostic x-rays (such as CT scans). These rays pass through the body and deliver radiation to the cancer tissues. This is not painful and you will not feel anything at the time of treatment. You may hear your hospital doctor or nurse talk about ‘fractions’ when they are discussing your treatment with you. Fractions is a word used instead of sessions. Sometimes a single fraction is all that is needed, but usually a number of fractions are given over the course of many weeks. The number of fractions you receive will be decided by your radiotherapy specialist.

Research into treatments for bile duct cancer is ongoing and advances are continuously being made. Clinical trials aim to assess and improve the success of treatments. Imperial College Healthcare NHS Trust is internationally recognised as centre of excellence for teaching and research. In the past, research carried out within the trust has contributed to advances in patient treatment in this and other hospitals. Liver cancer is specifically treated on the Hammersmith Hospital site, where the specialist liver teams are based. You may be asked to take part in a clinical trial. Your hospital doctor or nurse will discuss the treatment with you, after you have been given detailed written information, so that you have a full understanding of the trial and what it means to take part.

Sometimes experimental treatments are available before they are of proven benefit. These newer treatments require applications for ‘exceptional funding’ which can take many weeks to get considered, and may not necessarily be approved. This is also only if you are considered suitable and no other options are available. Experimental treatments currently available in this category include:

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Y-90 SIR Spheresare, a form of internal radiotherapy, delivered into your liver. This is only suitable if the cancer is contained within the liver and not spread out, so is not suitable for most bile duct cancers. This is to control and not cure the cancer.

Cyberknife is a form of external radiotherapy that can target smaller areas with more accuracy. It is only suitable in very few cases where the cancer has not spread and surgery is not possible. This is to control and not cure the cancer.

New drugs and devices are always being developed. Please discuss with your hospital doctor if all standard treatment options are not suitable.

During your diagnosis and treatment you are likely to experience a number of different emotions such as shock, disbelief, fear, anger etc. At times these emotions can be overwhelming and hard to control. It is natural and important to be able to express them. These feelings do not mean that you are not coping – they are part of the process that many people go through in trying to come to terms with their illness. Everyone has their own way of coping with difficult situations. Some people find it helpful to talk to family or friends, while others prefer to keep their feelings to themselves. There is no right or wrong way to cope, but help is available if you need it. Your clinical nurse specialist/key worker is here to support you if you would like to talk to a professional.

It is important to remember that there are people available to help you and your family.

(Macmillan) clinical nurse specialist in the hospital This is an expert nurse who has specialist training and experience in both cholangiocarcinoma and cancer nursing. He/she offers information and support during your illness. He/she can also advise on symptom control and refer you on to other services you may need.

Specialist cancer nurse in the community These nurses are specially trained to support people with cancer in their own homes. If needed a community specialist nurse can visit to help manage symptoms and answer any questions you may have. These nurses work closely with the hospital team and regularly update us on how you are getting on.

District nurse District nurses work closely with GPs and make visits to some patients at home if they need nursing care.

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Counsellors Some people need more than advice and support. They may find the impact of cancer leads to feelings of anxiety, helplessness or depression. Sometimes it is easier to talk to someone who is not directly involved in your illness, and specialist help in coping with these emotions is available. If you wish, your hospital doctor, nurse or GP can refer you to a counsellor who is an expert in the emotional problems of people with cancer. This service is also available to your loved ones.

Social worker If you feel that you need more help at home – for example, meals on wheels, help with washing or dressing, or a home help – let your doctor or nurse know so that they can arrange for you to see a social worker. This can take some time to do, so if you feel that you may need some help, please discuss your needs before they become a big problem to you.

Chaplain Some people find great comfort in religion and, particularly after a diagnosis of cancer, may have many questions. It could help to talk to a local minister or other religious leader. Hospital chaplains representing the major world faiths are appointed by the Trust. The chaplaincy service provides pastoral and spiritual support for patients, their relatives and friends. Please tell your nurse if you would like a chaplain of your faith to visit you while you are admitted.

Dietitian Many people have concerns about their diet when being treated for cholangiocarcinoma. Your sense of taste may change due to your treatment, or you may be anxious about your weight. If you have any concerns, speak to your teams, who may then involve a dietician, or give advice and provide supplements if needed.

Name: ___________________________________________________________ Email address:___________________________________@imperial.nhs.uk

Telephone: 020 3313 2019, Monday to Thursday (except bank holidays), between 08.00 and 16.00.

If your call is urgent, please contact your nurse via the bleep system by dialling Hammersmith Hospital switchboard number on 020 8383 1000 and asking for bleep 9332.

Telephone: 020 3383 1533, Monday to Friday (except bank holidays), between 08:30 and 16:30.

If your call is urgent, please contact your nurse via the bleep system by dialling Hammersmith Hospital switchboard number on 020 3383 1000 and asking for bleep 7845.

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The Cholangiocarcinoma Foundation This American non-profit organisation offers information to anyone affected by this disease. Website: www.cholangiocarcinoma.org/

Macmillan Cancer Support Helpline This is a free helpline for people affected by cancer who have questions about cancer, need support or just someone to talk to. It is open from Monday to Friday, 09.00–20.00 (interpretation service available). Telephone: 0808 808 0000

Information Prescription Service This service contains reliable and accurate cancer information to help patients manage their health more effectively. Website: www.nhs.uk/ips/

Benefits Enquiry Line The service provides advice and information on the range of benefits available. Telephone: 0800 88 22 00

Cancer Research UK This UK charity is dedicated to funding clinical research, providing information for patients, health professionals and the public and influencing public policy to keep cancer at the top of the health agenda. Helpline: 0808 800 4040 Website: www.cancerresearchuk.org/

Macmillan Cancer Information and Support Service at Charing Cross Hospital The information centre provides support, practical and signposting advice to anyone affected by cancer. The service is set in a confidential non-clinical environment in which people affected by cancer can discuss private and emotional needs. The centre is located on the ground floor of Charing Cross Hospital and is open (except bank holidays):

Monday and Friday, 09:00–17:00

Tuesday, Wednesday, Thursday, 09:00–16:00 Telephone: 020 3313 0171

Maggie’s Cancer Caring Centre Maggie’s is a cancer charity that creates places providing the emotional, practical and social support that people with cancer may need. The centre combines striking buildings, calming spaces, professional experts offering professional support, and the ability to talk and share experiences with a community of people who have been through similar experiences. Maggie’s West London is located in the grounds of

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Charing Cross Hospital but please note it is independent of our hospital. The centre is open Monday to Friday, 09:00–17:00. For more information please call 020 7386 1750.

We aim to provide the best possible service and staff will be happy to answer any questions you may have. If you were pleased with your care and want to write to let us know we would appreciate your time in doing so. However, if your experience of our services does not meet your expectations and you would like to speak to someone other than staff caring for you, please contact the patient advice and liaison service (PALS) on 020 3313 3322 for Charing Cross, Hammersmith, and Queen Charlotte’s and Chelsea Hospitals or 020 3312 7777 for St Mary’s and Western Eye Hospitals. You can also email PALS at pals@imperial.nhs.uk. The PALS team will listen to your concerns, suggestions or queries and are often able to solve problems on behalf of patients. Alternatively, you may wish to express your concerns in writing to: The chief executive Imperial College Healthcare NHS Trust Trust Headquarters The Bays, South Wharf Road London W2 1NY

This leaflet can be provided on request in large print, as a sound recording, in Braille, or in alternative languages. Please contact the communications directorate on 020 3312 5592.

Upper GI Published: Nov 2012

Review date: Nov 2015 Reference no: 2231T

© Imperial College Healthcare NHS Trust