Sarcomas are rare cancers that develop in the supporting tissues of the body. There are two main types: bone sarcomas and soft tissue sarcomas.

Bone sarcomas, such as osteosarcoma, can develop in any of the bones of the body. They may also develop in the soft tissue near bones.

Soft tissue sarcomas can develop in muscle, fat, blood vessels, and any of the other tissues that support, surround and protect the organs of the body.

This information should be read with our general information about bone cancer.

Primary bone cancers are cancers that start in the bone. They are rare, with only about 580 new cases each year in the UK. There are several different types of bone cancer. Osteosarcoma is one of the most common types.

Osteosarcoma usually develops in growing bones. Although it can occur at any age, it is most often found in teenagers and young adults and is slightly more common in males. Any bone in the body can be affected, but the most common sites are the arms or legs, especially around the knee joint.

There are several different types of osteosarcoma, including chondroblastic osteosarcoma. There are also rare subtypes, such as:

• parosteal
• periosteal
• telangiectatic
• small cell osteosarcoma.

Your doctor can tell you more about your type of osteosarcoma.

The exact causes of primary bone cancer are unknown. Osteosarcoma is thought to be related to periods of rapid bone growth, such as puberty. Adults who have a bone condition called Paget’s disease or who have previously been treated with radiotherapy may have a slightly increased risk of developing osteosarcoma. Rarely, some osteosarcomas may occur in people from families where there is an inherited faulty gene that increases the risk of developing several types of cancer, such as Li-Fraumeni syndrome.

Pain is the most common symptom of bone cancer. However, symptoms may vary depending on where in the body the cancer is and how big it is. Tumours that occur in or near joints may cause swelling or tenderness in the affected area.

Primary bone cancer is sometimes found when a bone that has been weakened by cancer breaks after a minor fall or accident.

Many things can cause these symptoms other than cancer. However, if you have bone pain, especially if it occurs at night, or any swelling, you should get it checked by your doctor.

You begin by seeing your family doctor (GP), who will check you and may arrange tests or x-rays. If your GP thinks you may have a bone tumour, they should refer you directly to a specialist hospital or bone tumour centre for more tests. Many of the tests for diagnosing bone tumours, such as bone biopsies, need to be done by an experienced team using specialist techniques.

The doctor at the hospital will ask you about your general health and any previous medical problems. They will check you and look at the painful area to check for any swelling or tenderness. You will probably have a blood test to check your general health.

You may need a variety of tests and investigations to diagnose an osteosarcoma. An x-ray of the painful part of the bone will usually identify a tumour, although sometimes they can be hard to see.

A small piece of the tumour will be removed and looked at under a microscope (a biopsy). You may have other tests to check whether the cancer has spread to other parts of the body. 

Bone x-ray

This involves the use of x-rays to build up a picture of the bone.

Biopsy

The doctor will take a sample of cells (a biopsy) to be checked by a pathologist (a doctor that specialises in cell types). If your doctor thinks you have bone cancer, the biopsy should be done at a specialist bone cancer centre.

Needle biopsy

A small sample of the tumour is taken from the affected bone using a needle. You will be given a local 

anaesthetic to numb the area. Sometimes a general anaesthetic is used.

Open or surgical biopsy

This type of biopsy is not often used, as the needle biopsy is much quicker and simpler. In an open biopsy, a small piece of bone is removed during a small operation while you are under a general anaesthetic. You may need this if a needle biopsy cannot be done or does not give a clear result.

Bone scan

This is a more sensitive test than an x-ray and shows up any abnormal areas of bone more clearly. A small amount of a mildly radioactive substance is injected into a vein, usually in your arm. 

Abnormal bone absorbs more radioactivity than normal bone so these areas are highlighted and picked up by the scanner as ‘hot spots’.

After you have the injection, you will need to wait 2 to 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.

MRI (magnetic resonance imaging) scan

This test uses magnetism to build up detailed pictures of your body. During the test, you will be asked to lie still on a couch inside a large metal cylinder that is open at both ends.

The test may take up to an hour. It can be slightly uncomfortable and some people feel a bit 

claustrophobic during the scan. It is noisy, but you will be given earplugs or headphones. You can hear and speak to the person operating the scanner.

If you have any metal implants (such as surgical clips or pacemakers), it will not be possible for you to have this test. In this situation, another type of scan may be used.

CT (computerised tomography) scan

In some people with osteosarcoma, the cancer may spread to the lungs. You may have a CT scan to check for this. The scan takes a series of x-rays that build up a 3D (three-dimensional) picture of the inside of the body. The scan is painless and takes just a few minutes. CT scans use small amounts of radiation, which is very unlikely to harm you and cannot harm anyone you come into contact with. 

You may be given a drink or an injection of dye that allows certain areas to be seen more clearly. This may make you feel hot all over for a few minutes. If you are allergic to iodine or have asthma, you could have a more serious reaction to the injection so it is important to let your doctor know before the scan.

You can normally go home as soon as the scan is over.

PET (positron emission tomography) scan or PET-CT scan

This uses low-dose radioactive sugar to measure the activity of cells in different parts of the body. A very small amount of a mildly radioactive substance is injected into a vein, usually in your arm. A scan is then taken a couple of hours later. Areas of cancer are usually more active than surrounding tissue and show up on the scan. After the injection, you will have to wait at least an hour before the scan. The scan takes 30 to 90 minutes. You can normally go straight home afterwards.

Usually, a scan that combines a PET scan and a CT scan (called PET-CT) is used, as it can give more information about the position and size of a tumour.

Waiting for test results can be an anxious time for you. It may help to talk about your worries with a relative or friend. You could also speak to one of our cancer support specialists.

Grading refers to the appearance of the cancer cells under a microscope. It gives an idea of how quickly a cancer may grow and develop. Low-grade means that the cancer cells look very much like normal cells. They are usually slow-growing and are less likely to spread. In high-grade tumours, the cells look very abnormal, are likely to grow more quickly, and are more likely to spread.

Most osteosarcomas are high-grade, but a type called parosteal osteosarcoma is usually low-grade. Another subtype (periosteal osteosarcoma) is usually treated as though it were high-grade.

The stage of a cancer is a term used to describe its size and whether it has spread beyond its original site. Knowing the type and the stage of the cancer helps the doctors plan the right treatment.

Most patients are grouped depending on if the cancer is found in only one part of the body (localised disease) or if it has spread from one part of the body to another (metastatic disease).

There are different staging systems used for bone cancer. One is the TNM staging system.

TNM stands for Tumour, Node and Metastases:

• T describes the size of the tumour.
• N describes whether the cancer has spread to lymph nodes.
• M describes whether the cancer has spread to another part of the body (called metastatic or sec-ondary cancer).

This information is sometimes put together, along with the grade, to describe a number system:

Stage 1A

The cancer is low-grade and less than 8cm across.

Stage 1B

The cancer is low-grade and either bigger than 8cm across or in more than one place in the same bone.

Stage 2A

The cancer is high-grade and less than 8cm across.

Stage 2B

The cancer is high-grade and bigger than 8cm across.

Stage 3

The cancer is high-grade and is in more than one place in the bone where it started.

Stage 4A

The cancer is of any grade and has spread to the lung.

Stage 4B

The cancer is of any grade. It has spread to lymph nodes or other parts of the body other than the lungs.

If the cancer comes back after treatment, this is called recurrent or relapsed cancer.

As osteosarcomas are rare, they are usually treated by a team of doctors and other healthcare professionals at a specialist hospital. This means that you may have to travel some distance to have treatment.

The type of treatment you have will depend on a few things, including the position and size of the cancer, whether it has spread, the grade of the cancer and your general health.

Most people with an osteosarcoma will need to have a combination of different treatments. The treatments that may be used are surgery, chemotherapy and radiotherapy.

Surgery is an important part of treatment and is used to remove the tumour in the bone. If surgery is not possible, radiotherapy may be used instead. Chemotherapy is used for most people with an osteosarcoma. It is often given to shrink the tumour before surgery.

Surgery

Major improvements have been made in surgery for bone cancer. In the past, the surgeon often needed to remove (amputate) the affected limb if osteosarcoma was found. Now they often remove just the affected part of the bone and some of the healthy tissue around it. The bone is then replaced with a specially-designed metal replacement (prosthesis) or a bone graft (bone taken from another part of the body). If the cancer affects a bone in or near a joint, the whole joint can often be replaced with an artificial one. This operation is called limb-sparing surgery.

Unfortunately, it is not always possible to use limb-sparing surgery and occasionally an amputation may be the only way to treat the cancer. This is often the case when cancer cells have spread from the bone into the nerves and blood vessels around it.

The type of surgery you have will depend on many different things. Your surgeon will talk about the different types of surgery before any decision is made about your treatment.

It is often helpful to talk to someone who has had the same operation you are going to have. The medical and nursing staff can arrange this. On some wards, a counsellor may be available to talk about any worries you may have.

Chemotherapy

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is an important treatment for most people with osteosarcoma as it can greatly improve the results of surgery. It is usually given before surgery and may shrink large tumours enough to avoid amputation. You will then have more chemotherapy after surgery to destroy any remaining cancer cells and to stop the sarcoma from spreading outside the bone. This is called adjuvant chemotherapy.

Children, teenagers and young adults (aged 30 or under) having adjuvant chemotherapy may also be given a course of a new drug called mifamurtide (Mepact^®). This may help to reduce the risk of osteosarcoma coming back. Your doctor can give you more information.

Chemotherapy can make you feel better by relieving the symptoms of the cancer, but it can have unpleasant side effects. Any side effects can often be controlled well with medicines.

Radiotherapy

Radiotherapy treats cancer by using high-energy rays to destroy the cancer cells, while doing as little harm as possible to normal cells. Radiotherapy is sometimes used after surgery (adjuvant radiotherapy) and may be used to treat the primary tumour (for example, where surgery is not possible).

Radiotherapy can cause side effects such as skin redness (erythema) and tiredness (fatigue). These side effects can be mild or more troublesome, depending on the strength of the radiotherapy dose and the length of your treatment. The radiotherapist can tell you what to expect.

Research into new ways of treating osteosarcoma is going on all the time. Treatments that are carried out on patients are called clinical trials. These are especially important for finding improvements in treatment for rare cancers. Your specialist can tell you more about trials that may be relevant to you.

Before any trial can take place, it must be approved by an ethics committee, which protects the interests of the people taking part.

Your doctor or a research nurse will talk about the treatment with you, so that you fully understand the trial and what it means to take part. You may decide not to take part or withdraw from a trial at any stage. You will still receive the best standard treatment available.

After your treatment is completed, you will have regular check-ups and x-rays. These will continue for several years. If you have any problems or notice any new symptoms in between these times, let your doctor know as soon as possible.

A small number of people may develop late side effects from the treatment they had, sometimes many years later. These can include a change in the way the heart and kidneys work and a slight increase in the risk of developing another cancer in later life. Your doctor or nurse will explain more about any possible late side effects.

You may have many different emotions, including anger, resentment, guilt, anxiety and fear. You may find yourself tearful, restless and unable to sleep. Or you may have feelings of hopelessness and depression. These are all normal reactions but it can be difficult and distressing to admit to them.

The need for support will vary from person to person and may depend on the treatment you have and any side effects this causes. Your specialist will tell you about any potential side effects and how to deal with them before you begin any treatment.

Some hospitals have their own emotional support services with trained staff, and some of the nurses on the ward will have had training in counselling. You may feel more comfortable talking to a counsellor out-side the hospital environment or to a member of your religious faith, if you are religious.

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. Our cancer support specialists can give you information and support to help you cope.

Organisations such as Sarcoma UK and Bone Cancer Research Trust can also provide information and support. Cancer52 represents many charities and works to improve the quality of life for people with rarer types of cancer.

Teenage Cancer Trust is a national charity providing specialist units, support, education and information for teenagers with cancer and their families.