Soft tissue sarcomas

The soft tissues of the body connect, surround and support other body tissue and organs. Soft tissue includes muscle, cartilage, fat, fibrous tissue, nerves and blood vessels.

These sarcomas develop from muscle and the surrounding tissue. There are two main types that affect younger people:

• Alveolar rhabdomyosarcomas – these are more common in teenagers and young adults. They can happen in any part of the body. But they are usually in an arm or leg (limb), or in the muscles of the chest or tummy (abdomen).
• Embryonal rhabdomyosarcomas – these are more common in children and can start in different areas. For example, they may start around the eye, or in the head and neck, bladder, womb or vagina.

This type of sarcoma usually happens in the tissue around the joints, like the elbow, knee or ankle. It can also happen in other parts of your body, but that is less common. They usually appear as hard lumps. This type of sarcoma might not be obvious straight away. This is because the lump grows slowly, and is not usually sore.

This type of sarcoma happens in the fibrous tissue in the body. Fibrous tissue joins the structures inside your body together. For example, it joins the muscles to the bones. Fibrosarcoma is most common in the arms, legs, chest or tummy, but it can also happen in other parts of the body.

Soft tissue Ewing's sarcoma is treated in the same way as Ewing’s sarcoma in a bone. You can read more about this in our bone cancer section.

We do not know what causes sarcomas in teenagers and young adults. Remember, nothing you have done has caused the cancer. People often think a knock or injury might have caused a sarcoma, but there is no evidence for this.

Some rare conditions that run in families (known as genetic conditions) can increase the chance of getting a soft tissue sarcoma. You might know if anyone in your family has had one of these conditions. 

Having radiotherapy as a child for another type of cancer may increase your risk of developing a sarcoma later. This would be from your teens onwards.

The symptoms of a sarcoma depend on where it is. It can be in the:

• arm or leg – symptoms include a lump or swelling, which is usually painless, but not always
• tummy – symptoms include a painful, swollen tummy, and problems going to the toilet to poo (constipation)
• bladder – symptoms include pain in the lower tummy, finding it difficult to pee (pass urine), and having blood in your pee
• chest – symptoms include breathlessness, a cough and pain in your chest
• head or neck – symptoms include a lump, a blockage and discharge from the nose or throat, or occasionally your eye may swell and stick out a bit.

You may also have other symptoms, such as:

• tiredness
• loss of appetite
• weight loss.

You should see your GP if you have any of these symptoms, or are worried you may have a sarcoma. They will examine you, and refer you to a hospital if they think you need to see a specialist doctor.

Remember, most people with these symptoms do not have a sarcoma.

You might have some tests when you visit your GP or at the hospital. The tests help the doctors see whether you have a soft tissue sarcoma.

Visiting your GP

If you think you might have some of the symptoms of a soft tissue sarcoma, you should visit your GP.

They will examine you, and usually arrange for some tests, such as an ultrasound scan. There may be other reasons why you are feeling unwell. So it can be difficult to diagnose the problem straight away. After these tests, your GP may refer you to a hospital for more tests. This could be a general hospital or a specialist cancer hospital.

If your GP thinks you have a soft tissue sarcoma, they may refer you directly to a sarcoma specialist.

At the hospital

You will probably see a lot of doctors, nurses and other staff. Your hospital doctor will examine you and arrange for some of the following tests:

• ultrasound scans
• x-rays
• bone scans
• MRI, CT or PET scans
• biopsies
• bone marrow tests.

You may not have all of these. After having some of these tests, you may be diagnosed with a sarcoma. If you are not already seeing a specialist, your hospital doctor will refer you to one.

More information about tests and scans

Further tests

If the tests show you have a sarcoma, you might need a few more tests to check your general health. These may include:

• blood tests
• tests to check your heart is healthy – this can include an echo-cardiogram (an ultrasound of the heart) or electro-cardiogram (ECG), or a nuclear medicine scan (MUGA scan)
• tests to check your kidneys are healthy – this can involve blood tests or having your pee (urine) collected for a short time or a nuclear medicine blood test (GFR).

This may seem like a lot of tests, but they help the doctors plan the best treatment for you.

Having tests and waiting for the results can be an anxious time. It can help to talk about how you are feeling. You can get support from family, friends and your specialist nurse and doctor. 

The three main types of treatment for soft tissue sarcomas are:

• surgery
• chemotherapy
• radiotherapy.

Most people have a combination of treatments. Your doctors will plan your treatment based on:

•  the type of sarcoma you have
• the stage of the cancer (how big it is and whether it has spread from where it started)
• the grade of the cancer (how fast the cancer cells are growing).

If you have any questions about your treatment, ask your doctor or nurse. They will be happy to help you understand what is involved.

Surgery to remove the cancer is one of the main treatments for soft tissue sarcomas. Your surgeon will aim to remove all of the cancer. They will also try to prevent any long-term effects as much as possible.

The operation you have will depend on the size of the sarcoma and where it is in your body. You might meet other people with the same type of sarcoma who have had a different operation. Some people will have an operation after another treatment, like chemotherapy. Your cancer doctor and specialist nurse will talk to you about your operation. They will help you understand what is involved and why you need to have it.

We have listed different types of operation below.

Wide local excision

You may have an operation called a wide local excision. This is to remove the cancer and an area of tissue surrounding it (called the margin). This tissue is checked to make sure it does not contain any cancer cells. Sometimes, the surgeon also removes some lymph nodes (glands) near the cancer. These are checked for cancer cells.

If there are cancer cells in the margin or lymph nodes, you may need to have more surgery.

Skin graft or soft tissue graft

If you have a large area of tissue removed, you may need to have a skin graft or soft tissue graft. This is when skin or muscle is taken from a different part of the body to place over where the sarcoma was. This is called plastic surgery or reconstructive surgery. This is to make sure your appearance is as natural as possible. It will also make sure you can still do the things you did before the operation. Both areas will slowly heal over a few weeks after the operation.

Limb-sparing surgery

If the sarcoma is in an arm or leg, you may have limb-sparing surgery. This means the surgeon takes away the cancer without removing the arm or leg. The surgeon removes all of the tumour from the limb. They also remove any bone and muscle the tumour is affecting.

The surgeon then replaces what they have taken away. This may involve using plastic surgery or reconstructive surgery to replace the tissue. If they need to remove any bone, they will replace it with a metal implant called an endoprosthesis.

If the cancer affects a bone in or near a joint, the surgeon can replace the joint with an artificial one.

Before limb-sparing surgery

If you have had chemotherapy, you will need 2 or 3 weeks to recover before you can have your operation. Your doctor will make sure you're well enough to have the operation. If you’ve lost weight or been off your food, you might need to put on a bit of weight. The hospital dietitian can help you do this. You will probably also see a physiotherapist, who will give you exercises before the operation to strengthen your muscles.

Ask your surgeon or specialist nurse if you have any questions about the operation.

After limb-sparing surgery

You will have a bandage or splint on your limb to keep it still and let it heal.

You may have a tube coming out of the wound. This is called a drain. It stops fluid building up. A nurse will take it out after about 2 to 4 days, or when there isn’t much fluid coming out.

If you don’t feel like eating or drinking much after your operation, you’ll have fluids given into a vein (drip).

You may feel a bit sore to start with, but you will have strong painkillers to control this. It is important to tell the nurses if you need extra pain relief. The pain usually starts to get better within 2 to 3 days. As time goes on, you will need fewer painkillers and will start feeling more comfortable. Be honest about how you are feeling to your doctors and nurses. They can make sure you are as comfortable and pain free as possible.

Once you’re home, you will probably feel a bit better and stronger every day. But if you do have any problems, it is important to get treatment for them straight away. If you don’t, they could become more serious.

If your new joint starts to stiffen up, you may need some more exercises. So tell your doctor and they can arrange this for you.

Tell your doctor straight away if you notice any new redness, soreness or swelling in the limb that’s been operated on. You might have an infection that needs treatment.

Amputation

Very occasionally, the only way to get rid of the cancer is by removing the limb where the sarcoma is growing. This is called an amputation, and it’s only ever done if there is no other choice.

Before the amputation

Your doctor will talk things through with you, so you fully understand what is involved. The thought of losing a limb can be frightening. It might help to talk to a specialist nurse or a counsellor about your feelings. It might also help to talk to someone who has had an amputation. Your nurse or doctor can arrange this. It's really important to get support at this tough time.

After the amputation

You will have bandages on the area that’s been operated on. There will be a drain coming from the wound to remove any fluid that builds up. A nurse on the ward will usually take the drain out after 2 to 4 days. Or they may do this when no more fluid is coming from the wound. You will also be given fluid into a vein in your arm until you feel like eating and drinking again.

You will probably feel sore after your operation, especially for the first 2 to 4 days. But you will have strong painkillers to help with this. It’s important to tell the doctors and nurses if you need extra pain relief. You may have pain that feels as if it’s coming from the part of the limb that has been removed. This is called phantom pain. Let your doctor or nurse know if you have this. There are medicines that can help ease it, or take it away. It usually gets better and goes away in time.

A physiotherapist will visit you after your operation. They will show you exercises to keep your muscles strong and flexible.

After having an arm removed, you will be able to get up and move around once you have recovered from the anaesthetic, and don’t feel too sore. If you have had a leg removed, the physiotherapist will show you how to move around in bed and get from your bed to a chair. You will probably be moving around with crutches or in a wheelchair within a few days.

Once you are ready, you will go to the hospital gym for physiotherapy. This is a big part of your recovery. Your physiotherapist will give you more exercises to do as your wound heals and you get stronger.

It will take a few months for your limb to heal to its final shape after the operation. So you will not be fitted with your permanent prosthesis (artificial limb) until this happens. In the meantime, you will get a temporary prosthesis. You will start learning how to use it in physiotherapy. A specialist team will help you recover from the operation, and help you get used to your temporary prosthesis.

You can usually go home about 5 days after your operation.

This is when you have anti-cancer drugs to destroy the cancer cells. For soft tissue sarcomas, you can have chemotherapy:

• before surgery – to shrink the tumour
• after surgery – to reduce the risk of the cancer coming back, and to treat cancer cells that have spread
• when it is not possible to remove the sarcoma with an operation.

For soft tissue sarcomas, chemotherapy is given into a vein, usually through a central line, a port or a PICC line. Our chemotherapy section has more information about how you have chemotherapy.

You have chemotherapy over a few days. Then you have a few weeks off, so your body can recover from any side effects. The days you have the chemotherapy and the rest period together are called a cycle. Most people have several cycles of chemotherapy.

You will probably need to stay in hospital for some of the time during chemotherapy. But you may be able to have part of your treatment as an outpatient.

Different chemotherapy drugs are used for soft tissue sarcomas. You usually have two or three drugs. Your cancer doctor and specialist nurse will tell you more about the type of chemotherapy you will have. They will also tell you how many cycles you need.

You may also have chemotherapy treatment as part of a clinical research trial. If your doctor thinks there is a suitable trial, they will talk to you about this. You will be able to talk things over with them and with your family before you decide what to do. It will be your decision whether or not to take part in a trial. We have more information about clinical research trials that is written for all ages.

Some of the main chemotherapy drugs for soft tissue sarcomas are:

• ifosfamide
• vincristine
• actinomycin D
• doxorubicin
• cyclophosphamide
• etoposide.

If you have a rhabdomyosarcoma, you may have ifosfamide, vincristine and actinomycin together. Giving these drugs together is called IVA.

Or you might have ifosfamide, vincristine actinomycin and doxorubicin. Giving these drugs together is called IVADO.

You may have different combinations of drugs to treat other types of soft tissue sarcoma. For example, you may have ifosfamide and doxorubicin, or vinorelbine and cyclophosphamide.

Side effects of chemotherapy

Different chemotherapy drugs cause different side effects. Some people have just a few side effects, and others have more. Your doctor or nurse can tell you more about what to expect. Most side effects are temporary and slowly disappear once treatment stops. But some may last longer or be permanent.

The most common side effects are:

• a sore mouth
• hair loss
• tiredness
• sickness
• being more at risk of getting an infection.

Radiotherapy uses high-energy x-rays to destroy cancer cells, while doing as little harm as possible to normal cells.

For soft tissue sarcomas you can have radiotherapy:

• after surgery – to reduce the risk of the cancer coming back in the same area, or to get rid of any cancer cells that may be left behind
• before surgery – to shrink the tumour and make it easier to remove with an operation when it is not possible to remove the sarcoma with an operation.

Radiotherapy is generally given every weekday (Monday to Friday). Each treatment takes a few minutes. The number of treatments will depend on the type, size and position of the sarcoma.

Side effects of radiotherapy

Radiotherapy is not painful, but it can cause some side effects. After a few treatments, you may feel tired. Depending on your skin type, your skin may also go red or get darker. It may also feel sore or itchy around the area being treated. This gets better once your radiotherapy treatment is over.

Other side effects depend on which part of your body is having treatment. Having radiotherapy around a joint can make it feel stiff, but physiotherapy can help.

This information is about life after having surgery for a soft tissue sarcoma. You may find it useful to read the information about surgery for a soft tissue sarcoma (above) first.  

Life after limb-sparing surgery  

The long-term effects of limb-sparing surgery depend on the part of your body affected and the operation you had. The best person to ask about your operation is your surgeon.  

After limb-sparing surgery, the limb might not work as well as it used to. Physiotherapy is an important part of your recovery. You will have exercises to do that help get your limb working as well as possible. These can be hard work, but it is important to keep going with them.  

Joint replacements

If you have had a joint replacement, you will be able to walk, swim and do most of the things you could do before. But your doctor might advise you not to play high-impact sports like hockey, football, tennis or rugby. These activities could damage or loosen the new joint. Check with your surgeon about any special instructions they have for what activities you can do. 

If you have had a shoulder replacement, you might find it harder to lift your arm above your shoulder. After any kind of joint replacement, you will have physiotherapy to help you get as much movement back as possible.  

Life after amputation  

After amputation, most people have an artificial limb (called a prosthesis). There are artificial limbs designed to let you do all types of physical activity. These activities include walking, swimming, running, cycling and playing sports.  

Different types of prosthesis let you do different things. Some people have one for wearing day to day, and an extra one specially designed for something like swimming or doing athletics.  

Sex and relationships might be something you worry about as soon as you find out you have cancer. Or you may only notice changes to your sex life as you go through treatment, or after it’s finished. We have information about the possible physical and emotional effects cancer can have on your sex life and relationships. 

If you have sex during cancer treatment, it is important to protect yourself and your partner. It is also important to prevent a pregnancy during this time. If you have any questions about this, ask your doctor or nurse for advice. We say more about this in our information about sex and relationships. 

Before you start cancer treatment, your cancer doctor or nurse will talk to you about your treatment options and the possible side effects. Some treatments can affect your fertility (ability to have a baby). We have more information about cancer treatment and fertility.

You might feel lots of different emotions. You might be tearful, angry, fed up or depressed at times. You might feel less confident, and that you'll never be able to live a normal life or be in a relationship. These feelings are normal, and it's important that you get support to help you cope with them.  

Other people's reactions can be also hard to cope with. You might worry about what your friends think, or how they're going to treat you. The thought of going back into school, college or work can be scary. Even people in your family who you are close to might not know what to say or do. Others might say things that sound stupid or seem hurtful. Most people find it helps to talk about how they are feeling. 

You could talk to:  

• your specialist nurse or keyworker 
• your family or friends  
• a counsellor – your doctor or nurse can arrange this 
• other young people in a similar situation, for example through a support group.  

When you finish your treatment, your doctor will talk to you about what happens next. They will explain some of the long-term risks that soft tissue sarcoma and its treatment could have for you. They will arrange to see you regularly in clinic to see how you are getting on.