A practical guide to understanding cancer. Understanding. Primary. bone. cancer

Transcription

1 A practical guide to understanding cancer Understanding Primary bone cancer

2 Contents 1 Contents About this booklet 2 1 The bones and primary bone cancer 5 2 Diagnosing primary bone cancer 19 3 Treating primary bone cancer 33 4 After your treatment 79 5 Your feelings and relationships 83 6 Work and financial support 93 7 Further information 99

3 2 Understanding primary bone cancer About this booklet This information is about primary bone cancer. We hope it answers some of your questions and helps you deal with some of the feelings you may have. Primary bone cancer is a cancer that starts in a bone. It s a rare cancer. About 600 people are diagnosed with it a year in the UK. Primary bone cancer is different from secondary bone cancer. Secondary bone cancer is a cancer that started elsewhere in the body and spreads to the bones. Secondary bone cancer is much more common that primary bone cancer. Your doctor will tell you whether your cancer is a primary or secondary bone cancer. We have more information about secondary bone cancer. Some types of primary bone cancer can affect children and young adults. This booklet is for adults and teenagers with primary bone cancer, and it s also for parents of children with the disease. We also have more information about osteosarcoma and Ewing s sarcoma in children, which we can send you. We can t advise you about the best treatment for yourself (or your child). This information can only come from your doctor, who knows your full medical history. At the end of this booklet are some useful addresses and websites (see pages ), and a page to fill in with your questions for your doctor or nurse (see page 120).

4 Contents 3 In this booklet we ve included quotes from people with primary bone cancer, which you might find helpful. Some are from healthtalkonline.org Others are from people who have chosen to share their experience with us by becoming a Cancer Voice. To find out more, visit macmillan.org.uk/cancervoices If you d like to discuss this information, call the Macmillan Support Line free on , Monday Friday, 9am 8pm. If you re hard of hearing, you can use textphone , or Text Relay. For non-english speakers, interpreters are available. Alternatively, visit macmillan.org.uk If you find this booklet helpful, you could pass it on to your family and friends. They may also want information to help them support you.

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6 1 The bones and primary bone cancer What is cancer? 6 The bones 8 Types of primary bone cancer 12 Risk factors and causes 14 Symptoms 16

7 6 Understanding primary bone cancer What is cancer? Cancer starts in cells in our body. Cells are tiny building blocks that make up the organs and tissues of our bodies. They divide to make new cells in a controlled way. This is how our bodies grow, heal and repair. Cells receive signals from the body telling them when to divide and grow and when to stop growing. When a cell is no longer needed or can t be repaired, it gets a signal to stop working and die. Cancer develops when the normal workings of a cell go wrong and the cell becomes abnormal. The abnormal cell keeps dividing making more and more abnormal cells. These eventually form a lump (tumour). Not all lumps are cancerous. Doctors can tell if a lump is cancerous by removing a small sample of tissue or cells from it. This is called a biopsy. The doctors examine the sample under a microscope to look for cancer cells. Normal cells Cells forming a tumour

8 What is cancer? 7 A lump that is not cancerous (benign) may grow but cannot spread to anywhere else in the body. It usually only causes problems if it puts pressure on nearby organs. A lump that is cancerous (malignant) can grow into nearby tissue. Sometimes, cancer cells spread from where the cancer first started (the primary site) to other parts of the body. They can travel through the blood or lymphatic system (see below). When the cells reach another part of the body, they may begin to grow and form another tumour. This is called a secondary cancer or a metastasis. We have a video on our website that explains how cancer develops. You can watch it on our website at macmillan.org.uk/aboutcancer The lymphatic system The lymphatic system helps protect us from infection and disease. It also drains lymph fluid from the tissues of the body before returning it to the blood. The lymphatic system is made up of fine tubes called lymphatic vessels that connect to groups of lymph nodes throughout the body. Lymph nodes (sometimes called lymph glands) are small and bean-shaped. They filter bacteria (germs) and disease from the lymph fluid. When you have an infection, lymph nodes often swell as they fight the infection.

9 8 Understanding primary bone cancer The bones The human skeleton is made up of more than 200 bones of different shapes and sizes. Skull Clavicle Ribcage Spinal column Pelvis Scapula Humerus Ulna Radius Femur Fibula Tibia The human skeleton

10 The bones 9 Bones are made of collagen (a type of protein) and minerals such as calcium and phosphate. These make bones strong and rigid. Bones do lots of important things: They support our bodies, and the joints at the end of bones act as levers so we can move around. They protect various parts of our bodies from injury, for example the ribs protect the heart and lungs. They store important minerals that are used by our bodies, such as calcium. Some bones are filled with a soft, spongy material called bone marrow, which makes blood cells. Spongy bone Bone marrow Compact outer shell The structure of a long bone

11 10 Understanding primary bone cancer Our bones are constantly being broken down and replaced with new bone. This helps maintain their strength and shape. There are two main types of cell that break down and replace bone: Osteoclasts break down and remove old bone. Osteoblasts make new bone. The joints at the end of bones are covered in cartilage. This is a tough, flexible material, like gristle. Because cartilage is more elastic than bone, it allows the bones to move freely at the joints. It also cushions the bones at the joints, to stop them rubbing against each other.

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13 12 Understanding primary bone cancer Types of primary bone cancer Primary bone cancer is rare. About 600 people are diagnosed with it in the UK each year. It can happen at any age and it s slightly more common in men than women. There are several different types of primary bone cancer. If your cancer is not one of the ones described here, contact our cancer support specialists on They will be able to give you more information about it. Osteosarcoma Osteosarcoma is the most common type of primary bone cancer. It s most common in teenagers, young adults and adults in their 60s, but people of any age can be affected. It can occur in any bone, but is most likely to develop around the knee, in the thigh bone (femur), in the shin bone (tibia) or in the upper arm (humerus). Ewing s sarcoma Ewing s sarcoma is named after the surgeon who first described it. It is more common in teenagers and young adults, but it can occur at any age. It s more likely to occur in young children than osteosarcoma is. Any bone can be affected, but the most common sites are the pelvis, thigh bone (femur) and shin bone (tibia). It s also possible for Ewing s sarcoma to start in the soft tissues of the body. This is called extraosseous Ewing s sarcoma (extra means outside, osseous means bone), or soft tissue Ewing s sarcoma.

14 Risk factors and causes 13 Chondrosarcoma Chondrosarcoma is usually a slow-growing tumour and is most common in middle-aged people. The cancer starts in cartilage cells (see page 10), although it can also grow inside a bone or on its surface. The most common places for it to develop are the upper arm (humerus) or thigh bone (femur), but it can develop in other bones, such as the ribs, pelvis or shoulder blade (scapula). Spindle cell sarcoma Spindle cell sarcoma is a rare type of bone cancer. It is similar to osteosarcoma, but normally occurs in adults over the age of 40. It s extremely rare in people under 20. When spindle cell sarcoma is in a bone, it s treated in a similar way to osteosarcoma. You may hear words like leiomyosarcoma or malignant fibrous histiocytoma (MFH) used to describe spindle cell sarcoma. Chordoma This is a rare cancer, which is normally slow-growing. It starts in the bones of the spine. It may be in the bottom of the spine (the sacrum), in the neck or in the bones at the bottom of the skull. It can occur at any age, but is more common in people over 40. Angiosarcoma Angiosarcoma can occur at any age. It usually affects the soft tissues, but rarely it can develop in bone. Angiosarcomas can affect any bone. They can develop in more than one bone at the same time, or in more than one place in a single bone. We can send you more information about these types of bone cancer.

15 14 Understanding primary bone cancer Risk factors and causes The exact causes of primary bone cancer are unknown. For most people with bone cancer, it s not clear why it has developed. Research into possible causes is going on all the time. Because a lot of bone cancers occur in teenagers and young people, it s thought that it may be related in some way to changes that happen when bones are growing. There are some factors that are known to increase a person s risk of developing primary bone cancer, which we discuss here. Previous radiotherapy or chemotherapy People who ve had high doses of radiotherapy to an area that includes the bones have a slightly increased risk of developing cancer in one of these bones. This is a very small risk, and most people who have radiotherapy never develop primary bone cancer. If it does occur, it s usually about years after having the radiotherapy. People who have previously been treated with certain chemotherapy drugs can have an increased risk of developing osteosarcoma. Non-cancerous (benign) bone conditions Having certain benign bone conditions can increase your risk of developing particular types of bone cancer. Paget s disease of the bone is a non-cancerous condition that causes painful and deformed bones. It can increase the risk of developing osteosarcoma. It mainly affects people over 60.

16 Risk factors and causes 15 A non-cancerous bone tumour called osteochondroma (or chondroma) can sometimes develop into chondrosarcoma. People with hereditary multiple exostoses (HME) have an increased risk of developing chondrosarcoma. HME is a rare condition that causes bony lumps to grow, most commonly in the arm or leg bones. It often starts in childhood and is usually, but not always, inherited. Inheriting a faulty gene Most bone cancers are not caused by an inherited faulty gene, but people with certain genetic conditions have an increased risk of developing bone cancer. People who have an inherited condition called Li-Fraumeni syndrome have an increased risk of developing osteosarcoma. Children who have retinoblastoma, a rare type of eye cancer caused by an inherited faulty gene, also have an increased risk of developing osteosarcoma. Injuries Sometimes people discover they have primary bone cancer after a knock to their bone. They may think the injury caused the cancer to develop. There isn t clear evidence that injury to a bone can cause bone cancer, but an injury may draw attention to a bone cancer that s already there.

17 16 Understanding primary bone cancer Symptoms These are the most common symptoms of primary bone cancer. Pain or tenderness in the area of the tumour This may start as an ache that doesn t go away. It may be made worse by exercise or feel worse at night. In children, this symptom may be mistaken for a sprain or growing pains. If a child or teenager has bone pain that persists during the night, it s always best to have this checked by their GP. Swelling around the affected area of bone Swelling may not show up until the tumour is quite large. It isn t always possible to see or feel a lump if the affected bone is deep within the body tissues. Leading up to my diagnosis, I developed occasional swelling and growing pains in my leg, particularly if I did any sort of exercise, such as walking or running for long periods of time. Tom

18 Symptoms 17 Reduced movement If the cancer is near a joint, this can make it more difficult to move the joint. It can affect the movement of the whole limb. If the affected bone is in the leg, it may cause a limp. If the tumour is in the spine, it may press on nerves, causing weakness or numbness and tingling in the limbs. Broken bone Bone cancer is sometimes found when a bone that has been weakened by cancer breaks spontaneously or after a minor fall or accident. This is called a pathological fracture. General symptoms Other symptoms may include tiredness, weight loss, and a high temperature or sweats. These symptoms aren t common but sometimes occur in people with Ewing s sarcoma. Many of these symptoms can be caused by other conditions that are more common than bone cancer. Because of this, it sometimes takes a long time for bone cancer to be diagnosed. Anyone with bone pain that lasts longer than a few weeks with no obvious cause should be referred to a bone specialist (orthopaedic doctor).

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20 2 Diagnosing primary bone cancer How bone cancer is diagnosed 20 Further tests 25 Grading and staging 30

21 20 Understanding primary bone cancer How bone cancer is diagnosed Usually you begin by seeing your family doctor (GP). They will examine you and arrange any tests or x-rays you need. Your GP may refer you to a local surgeon who specialises in bone diseases (an orthopaedic surgeon), or to a bone cancer specialist. If tests show that you may have primary bone cancer, you should be referred to a specialist hospital or bone tumour treatment centre (sarcoma unit). Children are referred to a children s (paediatric) hospital for some of their care. Teenagers may be referred to a teenage cancer unit. These units have specialist doctors with experience in diagnosing and treating young people with cancer. They also have a team of people to support teenagers. The specialist at the hospital or bone treatment centre will ask you about your symptoms. They will want to know about your general health and any previous medical problems. They will examine the affected area to check for any swelling or tenderness. You will have a blood sample taken to check your general health. You ll also have some of the tests listed over the following pages.

22 How bone cancer is diagnosed 21 Bone x-rays Bone x-rays may help show whether the cancer has started in the bone (primary bone cancer), or has spread into the bone from a cancer elsewhere in the body (secondary bone cancer). Sometimes the way the bone looks on an x-ray can help the doctor tell which type of bone cancer it is. This is often true for osteosarcoma. However, you will need other tests before the doctor can definitely say whether it s a primary or secondary bone cancer and which type of cancer it is. The photo below shows someone having a bone x-ray.

23 22 Understanding primary bone cancer MRI (magnetic resonance imaging) scan An MRI scan is used to assess the extent of the primary tumour so that the doctors can plan the best treatment. The photo below shows someone having an MRI scan. This test uses magnetism to build up a detailed picture of areas of your body. The scanner is a powerful magnet, so you may be asked to complete and sign a checklist to make sure it s safe for you. The checklist asks about any metal implants you may have, for example a pacemaker, surgical clips or bone pins. You should also tell the doctor if you ve ever worked with metal or in the metal industry, as very tiny fragments of metal can sometimes lodge in the body. If you do have any metal in your body, you probably won t be able to have an MRI scan. In this situation, another type of scan can be used.

24 How bone cancer is diagnosed 23 Before the scan you ll be asked to remove any metal belongings, including jewellery. Some people are given an injection of dye into a vein in the arm, which doesn t usually cause discomfort. This is called a contrast medium. It can help the images from the scan to show up more clearly. During the scan, you ll lie very still on a couch inside a long tube for about 30 minutes. It s painless but can be slightly uncomfortable, and some people feel a bit claustrophobic. It s also noisy, but you ll be given earplugs or headphones. You can hear, and speak to, the person operating the scanner during the scan. Bone sample (bone biopsy) A sample of bone is always needed to diagnose bone cancer. This is because x-rays and scans can t always show whether or not a tumour is cancer. If it is cancer, the bone biopsy should show which type of bone cancer it is. A bone biopsy is a specialised test. It should only be done by a radiologist or surgeon who s an expert in bone cancers. There are two ways of taking a bone biopsy: Core needle biopsy Before the biopsy, the doctor will give you an injection of local anaesthetic into your skin and around your bone to numb it. Then they will pass the biopsy needle through the skin into the bone to take the sample. They use a special needle to do this. They may take several samples. If the doctor can t feel the bone lump or if it s deep inside the body, the doctor may use an ultrasound or CT scanner (see pages 25 26) to help them guide the needle into the right place. You will usually be awake during a core needle biopsy, although you may be given a sedative to make you feel more relaxed and drowsy. Sometimes the biopsy is done under a general anaesthetic, particularly for children.

25 24 Understanding primary bone cancer For most people, a core needle biopsy will show whether the lump is cancer. However, sometimes it doesn t provide enough cells to give a clear diagnosis. In this case, you will need a surgical biopsy. Surgical biopsy This type of biopsy is done less often than a core needle biopsy. A surgeon uses a surgical knife (scalpel) to open the affected area and remove a sample from the lump. You will be given a local or a general anaesthetic before a surgical biopsy. This depends on your general health, the size of the tumour and how deep it is inside your body. The bone sample or samples are then sent to a specialist doctor (pathologist). The pathologist can tell whether the tumour is a cancer or not by examining cells from the sample under a microscope. If it is a cancer, your doctors may do further tests on the sample to find out which type of bone cancer it is. Waiting for test results Waiting for test results can be a difficult time. It may take from a few days to a couple of weeks for the results of your tests to be ready. You may find it helpful to talk to a partner, family member or close friend. Your specialist nurse or one of the organisations listed on pages can also provide support. You can also talk to one of our cancer support specialists on

26 Further tests 25 Further tests If your tests show that you have bone cancer, the doctor may want to do some further tests to see whether the cancer has spread outside the bone. CT (computerised tomography) scan Most patients with bone tumours will have a CT scan of their lungs. They might also have a CT scan of the affected bone. A CT scan takes a series of x-rays, which build up a three-dimensional picture of the inside of the body. The scan takes minutes and is painless. The photo below shows someone having a CT scan.

27 26 Understanding primary bone cancer The CT scan uses a small amount of radiation, which is very unlikely to harm you and will not harm anyone you come into contact with. You might be asked not to eat or drink for at least four hours before the scan. You may be given a drink or injection of a dye, which allows particular areas to be seen more clearly. This may make you feel hot all over for a few minutes. It s important to let your doctor know if you are allergic to iodine or have asthma, because you could have a more serious reaction to the injection. You ll probably be able to go home as soon as the scan is over. Bone scan This scan looks at all the bones in the body. It s more sensitive than an x-ray, and it shows up any abnormal areas of bone more clearly. A small amount of a radioactive substance is injected into a vein in your hand or arm. Abnormal bone absorbs more radioactivity than normal bone, so these areas are highlighted and picked up by the scanner as hot spots. The level of radioactivity used in the scan is very small and doesn t cause any harm to your body. After you have the injection, you will need to wait 2 3 hours before you have the scan. You may want to take a magazine, book or MP3 player with you to help pass the time. A bone scan is very similar to any other scan. It s not an unpleasant experience. Liesel

28 Further tests 27 If hot spots do show up on a bone scan, it isn t always clear whether they re caused by cancer or by other conditions, such as arthritis. Sometimes a CT scan (see pages 25 26) or MRI scan (see pages 22 23) may help the doctors decide whether changes on a bone scan are caused by bone cancer or by another condition. Some hospitals or treatment centres may do an MRI scan of the whole skeleton instead of a bone scan. This is to check for signs of cancer in any other bones away from the main tumour. Bone marrow sample You will only need this test if you have, or are likely to have, Ewing s sarcoma. Very occasionally, a Ewing s sarcoma can spread to the bone marrow (see page 9). The test involves a doctor taking a small sample (biopsy) of bone marrow from the hip bone. Taking a bone marrow sample

29 28 Understanding primary bone cancer Before the test, adults will be given a local anaesthetic injection into the area around the bone to numb it. Children will usually have a general anaesthetic. The doctor will then pass a special needle through the skin into the bone. When the needle is in position, the doctor will draw a small liquid sample from the bone marrow into a syringe. You may feel some discomfort when this is being done, but it should only last for a few seconds. You can have painkillers if you need them. The test is usually done in the outpatient department and takes about minutes. Sometimes the doctor needs to take a small core of bone marrow. In this case they will do a procedure called a trephine biopsy, which takes a few minutes longer than a bone marrow sample. A special type of needle is passed through the skin to the bone marrow. The needle has a tip that can cut out a sample of the bone marrow. You may feel bruised after the test and have an ache for a few days. Taking mild painkillers will help ease it. Sometimes the bone marrow and trephine biopsies are done at the same time. Your bone marrow samples will be sent to a laboratory to be looked at under a microscope. It may take 7 10 days to get the results. PET/CT scan This is a combination of a CT scan (see pages 25 26) and a PET (positron emission tomography) scan. A PET scan uses low-dose radiation to measure the activity of cells in different parts of the body. PET/CT scans give more detailed information about the part of the body being scanned. You may have to travel to a specialist centre to have one. You can t eat for six hours before the scan,

30 Further tests 29 although you may be able to drink. Before the scan, a mildly radioactive substance is injected into a vein, usually in your arm. The radiation dose used is very small. After having the injection you ll have to wait at least an hour before the scan. The scan usually takes minutes. You should be able to go straight home afterwards. Chest x-ray For people with primary bone cancer, the most common place for the cancer to spread to is the lung. A chest x-ray can show whether the lungs have been affected. Other tests If you re going to have chemotherapy (see pages 60 70), you will also have tests to check your hearing, kidneys and heart. Some chemotherapy drugs can affect how well you hear high-pitched sounds. So you may have hearing tests (audiograms) before and during your course of chemotherapy. To check how well your kidneys are working, you may have a small amount of mildly radioactive liquid injected into a vein in your hand or arm. This is carried through your kidneys then passed out in your urine. A few hours after the injection, a nurse will take blood samples from you. These show how well your kidneys are working. You may also have an electrical trace taken of your heartbeat (an ECG), an ultrasound scan of your heart (an echocardiogram), or a MUGA (multiple-gated acquisition) scan. A MUGA scan shows the movement of the heart and is used to check your heart function. If you need a MUGA scan, your doctors will tell you more about it.

31 30 Understanding primary bone cancer Grading and staging Knowing the grade and stage of the cancer affects the decisions you and your doctors make about your treatment. Grading Grading describes what the cancer cells look like under a microscope. The grade gives an idea of how quickly the cancer may develop. The most common grading system for primary bone cancer uses two grades: low-grade and high-grade. Low-grade means the cancer cells look very similar to normal bone cells. They are usually slow-growing and are less likely to spread. In high-grade tumours, the cells look very abnormal. They are likely to grow more quickly and are more likely to spread. All Ewing s sarcomas and most osteosarcomas and spindle cell tumours are high-grade.

32 Grading and staging 31 Staging The stage of a cancer describes its size and whether it has spread. The stages of primary bone cancer are also based on the grade of the cancer. There are two different staging systems used for primary bone cancer. This is the Enneking staging system, which is commonly used to stage primary bone cancers: Stage 1 The cancer is low-grade and hasn t spread beyond the bone. Stage 1 is divided into: Stage 1A The cancer is low-grade and is still completely inside the bone it started in. The cancer may be pressing on the bone wall and causing a swelling, but it has not grown through it. Stage 1B The cancer is low-grade and has grown through the bone wall. Stage 2 The cancer is high-grade and has not spread beyond the bone. Stage 2 is divided into: Stage 2A The cancer is high-grade and is still completely inside the bone it started in. Stage 2B The cancer is high-grade and has grown through the bone wall. Stage 3 The bone cancer may be any grade and has spread to other parts of the body, such as the lungs.

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34 3 Treating primary bone cancer Treatment overview 34 Surgery 43 Chemotherapy 60 Radiotherapy 71 Research clinical trials 76

35 34 Understanding primary bone cancer Treatment overview The treatments used for primary bone cancer are surgery, chemotherapy and radiotherapy. Most people need a combination of different treatments. Surgery (see pages 43 59) is a very important part of treatment. It is used to remove the tumour in the bone. Chemotherapy (see pages 60 70) is given to some people with primary bone cancer. Nearly everyone with a Ewing s sarcoma will have chemotherapy. It is also given to most people with osteosarcomas and spindle cell sarcomas. It s often given before surgery (called neoadjuvant chemotherapy). Giving chemotherapy before surgery helps to shrink the tumour, making it easier to remove. It can also be used after surgery (called adjuvant chemotherapy), when it s given to destroy any remaining cancer cells. Radiotherapy (see pages 71 75) is used to treat some types of bone tumour. It is usually given after surgery or chemotherapy. It may also be used where surgery isn t possible. Radiotherapy is particularly effective in treating Ewing s sarcoma. It s less effective for osteosarcoma, chondrosarcoma and spindle cell sarcoma, so it isn t often used for these types of bone cancer. However, it can help in certain circumstances. Specialist treatment centres Your treatment will usually be planned and given in a sarcoma treatment centre (sometimes called a sarcoma unit). This will either be one hospital or a group of hospitals that are close to each other and that work together. Sarcoma treatment centres specialise in treating people with primary bone tumours and soft tissue sarcomas.

36 Treatment overview 35 Primary bone cancer is rare and there are only a small number of sarcoma treatment centres in the UK. This means you may need to travel a long distance to reach one. Sometimes it s possible to have chemotherapy and radiotherapy treatments at a hospital closer to home. In this case, the treatments will be planned and given by a specialist cancer doctor (oncologist) who will work closely with the doctors at the sarcoma treatment centre. Teenage cancer units Some hospitals have teenage cancer units (sometimes called TYA units), which are specially designed for teenagers and young adults. Other hospitals may have special wards or areas for you if you re a teenager with cancer. There may be video games, DVDs and music to help you feel more at home.

37 36 Understanding primary bone cancer In a teenage cancer unit you may have access to a computer so that you can do some of your school or college work if you feel well enough. There may also be education specialists who can stay in touch with your school or college and support your learning needs while you re having treatment. Most teenage cancer units allow someone to stay with you. This is usually your parent or guardian but can also be a partner, friend or other family member, as long as they re over 16 and are well. In some hospitals there isn t a special ward for teenagers with cancer, so you are treated on an adult cancer ward. The staff on the adult cancer wards will still be able to look after you, although the wards may not have the same facilities as teenage cancer units. Children s cancer centres If your child has bone cancer, they will be treated in a hospital, or a part of a hospital, that specialises in diagnosing and treating children s cancers. These are often called principal treatment centres. These are relaxed and friendly places, and they aim to give you and your child a positive experience. You ll probably get to know the staff well. Almost all children s wards have facilities where parents can stay. The hospital will also have support staff, such as play therapists. Play therapists use play to help children cope with the experience of being ill. Children s cancer centres also have teachers, and most have education departments. The teachers at the hospital will contact the teaching staff at your child s school to make sure they can continue their education whenever they feel well enough. It s even possible for children to take exams in hospital if needed.

38 Treatment overview 37 If your child leaves hospital but isn t able to go back to school yet, tutoring can often be arranged with the local education authority. The person responsible for your child s schooling while they are in hospital will be able to organise this for you. How treatment is planned In most hospitals, a team of specialists will talk to you about the treatment they feel is best for your situation. This multidisciplinary team (MDT) will include: a surgeon (who specialises in bone cancers) a medical oncologist (a chemotherapy specialist) a clinical oncologist (a radiotherapy and chemotherapy specialist) a nurse specialist radiologists (who help analyse x-rays and scans) pathologists (who advise on the type and extent of the cancer) a paediatric oncologist (a doctor who specialises in treating children with cancer) if appropriate a keyworker, who will be your main contact and will make sure you get help and support throughout your treatment this may be a specialist nurse or other healthcare professional, such as a physiotherapist. It may also include other healthcare professionals, such as a dietitian, physiotherapist, occupational therapist (OT), psychologist or counsellor.

39 38 Understanding primary bone cancer The MDT will take a number of factors into account when advising you on the best course of action, including your general health, the type and size of the tumour, and whether it has begun to spread. If two treatments are possible for your type and stage of bone cancer, your doctors may offer you a choice of treatments. Sometimes people find it hard to make a decision. If you re asked to make a choice, make sure you have enough information about the different options, what s involved and the possible side effects. This will help you decide on the right treatment for you. Many people with primary bone cancer will also be offered the opportunity to take part in a clinical research trial (see pages 76 77). These trials help doctors find new and better treatments for people with primary bone cancer. If your doctor thinks you are suitable for a clinical trial, they will discuss this with you. Remember to ask questions about any aspects of your treatment you don t understand or feel worried about. It may help to discuss the benefits and disadvantages of each option with your cancer specialist, keyworker or specialist nurse. You can also talk to our cancer support specialists on If you have any questions about your treatment, don t be afraid to ask your doctor or nurse. It often helps to make a list of questions and to take a relative or close friend with you to appointments.

40 Treatment overview 39 The benefits and disadvantages of treatment Many people are frightened by the idea of having cancer treatments, particularly because of the side effects that can occur. However, these can usually be controlled with medicines. Treatment can be given for different reasons, and the potential benefits will vary depending on your individual situation. In people with early-stage bone cancer, treatment is often given with the aim of curing the cancer. You may also be given additional treatments to reduce the risk of it coming back. If the cancer is advanced and has spread to other parts of the body, treatment may only be able to control it, improving symptoms and quality of life. However, for some people in this situation the treatment will have no effect on the cancer, and they will get the side effects without any of the benefit. If you ve been offered treatment that aims to cure the cancer, deciding whether to accept it may not be difficult. However, if a cure is not possible and the purpose of treatment is to control the cancer for a period of time, it may be more difficult to decide whether to go ahead. Making decisions about treatment in these circumstances is always difficult, and you may need to discuss in detail with your doctor whether you want to have treatment. If you choose not to have it, you can still be given supportive (palliative) care, with medicines to control any symptoms.

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42 Treatment overview 41 Giving your consent Before you have any treatment, your doctor will explain its aims. They will usually ask you to sign a form saying that you give permission (consent) for the hospital staff to give you the treatment. If you re the parent of a child under 16 who needs treatment for bone cancer, the doctor will ask you or your child to sign the consent form. The law about children consenting to treatment is very complicated, so make sure you discuss any concerns you have with the doctor. No medical treatment can be given without your consent, and before you are asked to sign the form you should be given full information about: the type and extent of the treatment its advantages and disadvantages any significant risks or side effects any other treatments that may be available. If you don t understand what you ve been told, let the staff know straight away, so they can explain again. Some cancer treatments are complicated, so it s not unusual to need repeated explanations. It s a good idea to have a relative or friend with you when the treatment is explained, to help you remember the discussion. You may also find it useful to write a list of questions before your appointment. You could use the form on page 120.

43 42 Understanding primary bone cancer People sometimes feel that hospital staff are too busy to answer their questions, but it s important for you to know how the treatment is likely to affect you. The staff should be willing to make time for your questions. You can always ask for more time if you feel you can t make a decision when your treatment is first explained to you. You are also free to choose not to have the treatment. The staff can explain what may happen if you don t have it. It s essential to tell a doctor or the nurse in charge, so they can record your decision in your medical notes. You don t have to give a reason for not wanting treatment, but it can help to let the staff know your concerns so they can give you the best advice. Second opinion Your multidisciplinary team uses national treatment guidelines to decide on the most suitable treatment for you. Even so, you may want another medical opinion. If you feel it will be helpful, you can ask either your specialist or GP to refer you to another specialist for a second opinion. Getting a second opinion may delay the start of your treatment, so you and your doctor need to be confident that it will give you useful information. If you do go for a second opinion, it may be a good idea to take a relative or friend with you, and have a list of questions ready, so you can make sure your concerns are covered during the discussion.

44 Surgery 43 Surgery Surgery is one of the main treatments for primary bone cancer. Experienced surgeons who work in a specialist treatment centre will do the surgery. The aim is to remove the tumour from the bone while trying to keep as much function as possible. It s usually possible to remove the affected part of the bone and replace it with a custom-made metal fitting (endoprosthesis), or with a bone from another part of the body (bone graft). If the cancer affects a bone in or near a joint, the whole joint can often be replaced with an artificial one. These operations are called limb-sparing surgery. In some cases, the surgeon can remove the affected bone without replacing it with anything and without it affecting the way you move. It is not always possible to use limb-sparing surgery, and occasionally the surgeons need to remove (amputate) a whole limb. This is to make sure the cancer has been completely removed. It may also be needed if your movement will be better after an amputation than after limb-sparing surgery. Most bone tumours affect the limbs or pelvis. The surgery to these areas will usually be done by an orthopaedic surgeon. If the tumour is in a less common place, such as the bones of the face, skull or chest, the sarcoma team will work with surgeons who specialise in those areas. The type of surgery you have will depend on a number of factors. Your surgeon will discuss the different types of surgery in detail with you before you make any decision about your treatment.

45 44 Understanding primary bone cancer Limb-sparing surgery Before surgery, your doctor will explain the procedure to you, to make sure you fully understand what s involved. You can ask questions about how the surgery might affect you. You may find it helpful to talk to someone who has had the same operation. The medical staff or your keyworker may be able to arrange this for you. Some hospitals have a counsellor you can talk to about your feelings and any worries you may have before the operation. Some people like to see the replacement part or endoprosthesis (often just called a prosthesis) that will be used during their operation. If you would like to see it, your surgeon or keyworker can usually arrange it for you. A physiotherapist will talk to you before your operation. They may give you some exercises to do before surgery, to help strengthen your muscles. If you ve had neoadjuvant chemotherapy (see page 34), it usually takes a couple of weeks before your blood cell levels get back to normal and you re ready to have your operation. After your operation At first your limb will be firmly bandaged, or you may have a splint in place to keep it still. This will give the bone graft, or artificial joint or bone, time to start joining firmly on to the rest of the bone in the limb. You will probably have a drainage tube in the wound, to remove any fluid that collects in the area of the operation. The fluid and blood drains into a small container attached to the other end of the tube. Drains are usually taken out after 3 4 days. You will also have stitches or staples to close the wound. These are usually taken out about days after the operation.

46 Surgery 45 Eating and drinking For the first few hours after your operation, you probably won t feel like eating or drinking much, so you ll be given fluids as a drip into a vein in your hand or arm (an intravenous infusion). A nurse will take it out once you start eating and drinking again. Pain After your operation you ll need pain-killing drugs for a few days. These may be given into a vein (intravenously), into the space around your spinal cord (epidural), into a muscle (intramuscularly) or as tablets. Pain can usually be well-controlled with painkillers. To start with, you will probably need a strong painkiller such as morphine. You may be given intravenous pain relief through a syringe connected to an electronic pump. The pump can be set to give you a continuous dose of painkiller. You may also have a handset with a button you can press if you feel sore. This is called patient-controlled analgesia (PCA). It s designed so that you can t have too much painkiller (an overdose), so it s fine to press it whenever you re uncomfortable. If you ve had surgery on your leg, you may have pain relief using an epidural. A fine tube is put in through your back into the area just outside your spinal cord, called the epidural space. A local anaesthetic can be given continuously into this space to numb the nerves that run to your legs. Let your nurses and doctors know as soon as possible if you re in pain. This will help them give you the combination and dose of painkillers that s right for you.

47 46 Understanding primary bone cancer Physiotherapy Physiotherapy is a very important part of your recovery. It helps you regain muscle strength and get good movement back in your limb. A physiotherapist will come to see you soon after your operation and show you some exercises to do. These can be hard work, but it s important to keep going with them, as they will help you recover. Physiotherapy exercises If you have an operation on your leg or pelvis, you will need to use walking aids such as a frame or elbow crutches to help you move around for some time after the surgery. A physiotherapist will teach you how to get in and out of bed and how to safely use the walking aid to go home.

48 Surgery 47 You may need to continue to have physiotherapy as an outpatient for some time after your operation. After my operation, my medical team were very keen on me doing exercises to strengthen the prosthesis. These included bending, stretching and strengthening. It was hard work while I was having treatment, but when I look back now I realise how important it was. It still helps me now, seven years later. I still have regular physiotherapy sessions and attend physiotherapy rehabilitation exercise classes. They continue to help me regain muscle strength, balance and confidence. Katie Going home You ll usually be able to go home once your wound has healed and you can move around safely. For most people, this is about 7 10 days after the operation. Most people recover well after their surgery and are able to move around quite soon. Some people take longer to recover and need extra help. Before you go home, the staff will talk to you about your home situation. If you live alone or have several stairs to climb, you may need some help managing at home. If you have any worries about going home, make sure you discuss them with the nursing staff so they can organise some help.

49 48 Understanding primary bone cancer Living with limb-sparing surgery After limb-sparing surgery, people are often able to do most of the things they could before, including taking part in exercise and sports. But, depending on which limb was affected, there may be some things you can t do or that you find harder. Your surgeon can explain to you the particular risks of the operation you re having, how well the limb will work afterwards and the risk of possible complications, such as infection. Knee joint When the knee joint is replaced, the new joint normally works very well. It s fine for you to go swimming or cycling after surgery, but your doctor might advise you against doing high-impact sports like hockey, football, tennis or rugby. This is because of the risk of damaging or loosening the joint. Some people find that, years after their surgery, their prosthetic knee joint may loosen or cause pain. If this happens, further surgery will be needed. Hip joint Replacing a hip joint is usually very successful. Young people will have good strength in the new hip, but older people will usually need to use a walking stick. This is because their joint and muscles may not be as strong as they were before the operation. Hip replacements may also loosen, and some people will need to have further surgery within 10 years of having the hip replaced. Shoulder joint People who have a shoulder replacement can normally move their arm around very well below shoulder height. However, they re usually not able to raise their arm above shoulder height. It s uncommon for a shoulder joint prosthesis to loosen. They generally last for many years and cause few problems.

50 Surgery 49 My tibia was replaced with a metal prosthesis. It left me with a slight limp, which limits my walking. I have been issued with a Blue Badge, enabling me to use disabled parking. This is extremely useful, because it minimises the distance I have to walk. Tom

51 50 Understanding primary bone cancer Infection With any prosthesis, the main problem that can occur is infection. If the prosthesis gets infected, it will need to be taken out and replaced. The area needs to be cleaned completely with antibiotics before a new replacement joint can be put in. Bone grafts Where limb-sparing surgery is on a straight part of a bone, it may be replaced by a piece of bone taken from another area of the body. This is known as a bone graft. The main problem that can occur with these operations is an infection in the replacement bone. Children and teenagers If you have a prosthesis put into your limb while you re still growing, it will need to be lengthened as the leg or arm grows. Some types of prosthesis are lengthened during further surgery. Other prostheses can be lengthened from outside the limb over a couple of years, to allow for continuing growth. These prostheses are called non-invasive growers. Your specialist or keyworker will explain how your prosthesis will be lengthened. There s a limit to how much a prosthesis can be lengthened, so younger children will need to have their original prosthesis replaced with a longer one when they re older. If you have a bone graft, the limb may not grow normally. In this case, you will need further surgery to keep the limb the same length as the unaffected limb.

52 Surgery 51 Amputation It s not always possible to do limb-sparing surgery, and occasionally the surgeon will need to remove (amputate) the limb. This may be because the cancer has spread from the bone into the surrounding blood vessels. You may also need an amputation after limb-sparing surgery if there s an infection in the bone that persists despite treatment, or if the cancer comes back in the bone. Very occasionally, after discussion with their specialist doctor and family, people choose to have an amputation instead of limb-sparing surgery. The preparation for amputation is similar to the preparation for limb-sparing surgery (see page 44). You may find it helpful to talk to the rehabilitation team before you come in for surgery. They will answer any questions or concerns about how you will manage after the operation. Psychological support for people who are about to have an amputation is also important. Some hospitals and prosthetic rehabilitation units have a counsellor you can talk to about your feelings and any worries you may have before the operation. It may also help to talk to someone who has had the same operation and can give you practical advice as well as support. The nursing and therapy staff may be able to arrange this for you. After your operation You probably won t feel like eating or drinking much for the first few hours after your operation, so you ll be given fluids into a vein in your hand or arm (an intravenous infusion). A nurse will take it out once you start eating and drinking again. You may also have an oxygen mask on when you first wake up. Some people will have a tube in their bladder (catheter) to drain urine into a bag until they are up and moving around.

53 52 Understanding primary bone cancer The part of your body that has been operated on will be covered with bandages or a plaster cast to control swelling. There will be a tube (drain) coming from your wound to drain off excess blood and fluids into a small container. This will usually be taken out after 3 4 days. You will also have stitches or staples to close the wound. These are usually taken out about days after the operation. Pain You may have some discomfort for a few days after your operation. To start with, you may have morphine through an intravenous patient-controlled analgesia (PCA) pump, or you may have an epidural infusion (see page 45). It s important to let the nurses know if you re still in pain so they can adjust your painkillers. Phantom pain After your surgery you may have pain that feels like it s coming from the part of the limb that has been amputated. The pain often feels like a cramping, stabbing or burning sensation. It is a type of nerve pain known as phantom pain. You will have some medication at the time of surgery to reduce the risk of this happening. Most people find that phantom pain gets better with time and eventually goes away, but some people find that the pain can affect them long-term. Other ways of managing phantom pain are acupuncture or graded motor imagery, which includes visualisation techniques and mirror therapy. You can talk to your physiotherapist or occupational therapist about these treatments. Your doctor can advise you on the best painkillers to take if you have phantom pain. They include painkillers such as morphine, and other medications such as anti-depressants and anti-convulsants that work by reducing nerve pain.