Introduction

This section is for teenagers and young adults. It’s about a type of cancer called soft tissue sarcoma.

There are several different types of soft tissue sarcoma. The information in this section covers rhabdomyosarcomas, synovial sarcomas, soft tissue Ewing’s sarcoma and fibrosarcomas. These are the most common types in teenagers. If you have a different type and want to know more, contact Macmillan.

|If you're looking for information about soft tissue sarcomas in people of all ages, please see our general soft tissue sarcoma section.

Soft tissue sarcomas are cancers that develop from cells in the soft, supporting tissues of the body. There are many different types, but the ones that tend to affect teenagers are:

  • rhabdomyosarcomas
  • synovial sarcomas
  • soft tissue Ewing’s sarcoma
  • fibrosarcomas

Sarcomas can also develop in the bones. This is called osteosarcoma. You can find out more in Macmillan's bone cancer section.

Signs and symptoms of soft tissue sarcomas

The symptoms of a sarcoma will depend on where it is:

  • if it’s in an arm or leg, symptoms include a lump or swelling in the limb. This is usually painless, but not always.
  • if it’s in the tummy (abdomen), symptoms include a painful, swollen tummy, and problems going to the toilet to poo (called constipation).
  • if it’s in your bladder, symptoms include pain in the lower tummy, finding it difficult to wee (pass urine), and having blood in your wee.
  • if it’s in the chest, symptoms can include breathlessness, a cough and pain in your chest.
  • if it’s in the head or neck, symptoms include a lump, a blockage and discharge from the nose or throat. Occasionally an eye may become swollen and stick out a bit.

You may also have other symptoms, such as tiredness, loss of appetite or weight loss.

Remember - most people with the symptoms listed here won’t have a sarcoma.  But if you have any of these symptoms, or are worried that you may have a sarcoma, the first thing to do is to see your GP. They'll examine you and refer you to a hospital if they think you need to see a specialist doctor.

Causes

We don't know what causes sarcomas in teenagers and young adults.

People often think a knock or injury might have caused a sarcoma, but there’s no evidence for this.

Remember that nothing you’ve done has caused the cancer.

Types of soft tissue sarcoma

Rhabdomyosarcoma

This type of sarcoma grows in the active muscles of the body. These are muscles that we have control over, like the muscles we use to move our arms or legs. Rhabdomyosarcomas are most likely to happen in the head, neck, bladder, vagina, arms, legs and trunk. 

There are different types or rhabdomyosarcoma. The most likely one to happen in teenagers and young adults is called an alveolar rhabdomyosarcoma and affects an arm or a leg. 

To find out more look at Macmillan's rhabdomyosarcoma information that is written for all ages.

Synovial Sarcoma

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

Soft tissue Ewing’s sarcoma

If you have a type of soft tissue sarcoma called a soft tissue Ewing's sarcoma, it's treated in the same way as a Ewing’s sarcoma in a bone. You can read more about this in Macmillan's bone cancer section.

Fibrosarcoma

This type of sarcoma happens in the fibrous tissue in your body. Fibrous tissue joins the structures inside your body together - like your muscles to bones. You’re most likely to find a fibrosarcoma on your arms, legs or trunk, but they can happen deeper in your body.    

We also have more info about:

If you're looking for information about soft tissue sarcomas in people of all ages, see our general soft tissue sarcoma information.

The soft tissue

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.

Types of soft tissue sarcoma

Soft tissue sarcomas are cancers that develop from cells in the soft, supporting tissues of the body.

There are many different types, but the ones that tend to affect teenagers are: 

  • Rhabdomyosarcomas 
  • Synovial sarcomas 
  • Fibrosarcomas

There are also bone sarcomas. You can find out more about these in Macmillan's bone cancer section.

Doctors can tell the type of soft tissue sarcoma by closely examining the sample of tissue that’s been removed (called a biopsy) under a microscope.

Rhabdomyosarcomas

These 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 and are usually found 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 a number of areas, for example, around the eye, where the throat joins with the nose, the bladder, the womb, or the vagina. 

Synovial sarcomas

These usually develop near to but not inside joints, such as the knee or elbow. But they can develop anywhere in the body. They usually appear as hard lumps.

Fibrosarcomas

These start in cells called fibrocytes, which form the tissue that helps join parts of our body together, such as joining the muscles to the bones. Fibrosarcomas are usually found in the arms, legs or torso, but can also start in areas deeper in the body.

The information in this section covers the common types of soft tissue sarcoma, but if you have a different one and want to know more, please contact Macmillan.  

Causes

We don't know what causes sarcomas in teenagers and young adults.

Remember that nothing you’ve done has caused the cancer. 

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

People who had radiotherapy for another type of cancer when they were a child may have an increased risk of developing a sarcoma in later life - this would be from their teens onwards. 

People often think a knock or injury might have caused a sarcoma, but there’s no evidence for this.

We also have more info about:

If you're looking for information about soft tissue sarcomas in people of all ages, see our general soft tissue sarcoma information.

Tests for soft tissue sarcomas

There are some tests you might have at your GP surgery or at the hospital. These tests will 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 go to your GP. They'll be able to talk to you about your symptoms, and they can arrange tests to find out more.

They’ll examine you and usually arrange for some tests, such as x-rays. There may be a number of reasons why you're feeling unwell, so it can be difficult to diagnose the problem straight away. If your GP can't find the cause, you'll be referred to a specialist at the hospital.

At the hospital

To begin with you may see a specialist at a general hospital for more tests. If your GP suspects you have a soft tissue sarcoma, they may refer you directly to a sarcoma specialist.

If you're diagnosed with a sarcoma and you’re not already seeing a specialist, you’ll be referred to one. You'll probably see a lot of doctors, nurses and other staff.

Your hospital doctor will examine you and arrange for some of the following tests, which Macmillan have information on:

You won't necessarily need all of these tests.

Further tests

If the tests show that you have a sarcoma, you might need a few more tests to check how your body is working in general. Macmillan have information on these, including:

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

Having tests and waiting for the results can be a scary time. Talking about how you feel and getting support from family, friends and your specialist nurse and doctor can make it a bit easier.  You can also use Macmillan's online community for young people that have had similar experiences or are going through the same as you.  

We also have more info about:

If you're looking for information about soft tissue sarcomas in people of all ages, see our general soft tissue sarcoma information.

Treating soft tissue sarcomas

This section is for teenagers and young adults. It’s about treatment for a type of cancer called a soft tissue sarcoma. There are different types of soft tissue sarcoma, and the information in this section covers rhabdomyosarcomas, synovial sarcomas and fibrosarcomas, which are the most common types in teenagers.

If you have a different type and want to know more, please contact Macmillan.

The three main types of treatment for soft tissue sarcomas are surgery, chemotherapy and 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-growing the cancer cells are).

If you have any questions about your treatment, ask your doctor or nurse. They’ll be happy to help you understand what's involved.

If you have a type of soft tissue sarcoma called a soft tissue Ewing's sarcoma, it's treated in the same way as a Ewing’s sarcoma in a bone. You can read more about this in Macmillan's bone cancer section.

Chemotherapy (chemo)

This is when you're given anti-cancer drugs to destroy the cancer cells. For soft tissue sarcomas, chemo can be given in a number of situations:

  • 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 isn’t possible to remove the sarcoma with an operation.

For soft tissue sarcomas, chemo is given into a vein, usually through a central line, a port or a PICC line. You can find out more about chemo and how it's given.

You’ll have chemo over a few days, then you’ll have a few weeks off so that your body can recover from any side effects. The days you have the chemo and the rest period afterwards are called a cycle. Most people have several cycles of chemo.

You'll probably need to stay in hospital for some of the time during chemo, but it’s often possible to have part of your treatment as an outpatient.

Different chemo drugs are used for soft tissue sarcomas, and you’ll usually have two or three drugs in combination. This is sometimes called a regimen or treatment plan. Your doctor will decide which regimen or treatment plan is best for you depending on the grade and stage of your sarcoma. You may have chemotherapy treatment as part of a clinical research trial.  It will be your decision whether or not you take part in a trial.  Your doctor will explain it fully before you make the choice if there is a trial that is right for you.

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

  • ifosfamide
  • vincristine
  • actinomycin D
  • doxorubicin
  • cyclophosphamide
  • cisplatin

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

You might also be given ifosfamide, vincristine actinomycin and doxorubicin. Giving these drugs together is called IVADO.

Macmillan have more information about chemotherapy, including information about individual drugs and combinations of drugs, IVA and IVADO chemotherapy. This is written for anyone who's looking for information about chemotherapy, not just for young adults.

Other types of soft tissue sarcoma can be treated with different combinations of drugs, such as ifosfamide and doxorubicin, or vinorelbine and cyclophosphamide. Your cancer doctor and specialist nurse will explain more about the type of chemotherapy you’re going to have and how many cycles you’ll need.

Side effects

Different chemo drugs cause different side effects. Some people have just a few side effects and others have more. It's hard to know how it's going to be for you, as everyoneis different. The most common are hair loss, tiredness, sickness and being more at risk of getting an infection.

Side effects can often be controlled, so always let your doctor or nurse know about the side effects you’re having. They can usually do something to improve them. Most side effects are short-term (temporary) and gradually disappear once treatment stops.

If you want to know more about the type of chemo you’re having and its side effects, there’s lots more information in Macmillan's chemotherapy section.

Surgery

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, and at the same time reduce 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. Everyone is different. Your cancer doctor and specialist nurse will talk to you about your operation and make sure you understand why you need to have it and what's involved. Below are some of the different types of operation you might have for a soft tissue sarcoma.

We also have more information about what will happen before and after the operation in our surgery section.

Wide local excision

You may have an operation called a wide local excision to remove the cancer and an area of tissue surrounding it (called the margin). This tissue is checked to make sure it doesn’t contain any cancer cells. Sometimes the surgeon also removes some of the lymph nodes (glands) near the cancer to find out if there are any cancer cells there. If there are cancer cells in the margin or lymph nodes, some people may need to have more surgery.

Skin graft or soft tissue graft

If you need to have a large area of tissue removed, you may need to have a skin graft or soft tissue graft. This is when skin and sometimes muscle is taken from a different part of the body to place over the area where the sarcoma was removed from. This is called plastic surgery or reconstructive surgery. It’s done to give you as natural an appearance as possible and to make sure you can still do the things you did before your operation. Both operation sites will gradually 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. You’ll have all of the tumour taken away from your limb, as well as any bone and muscle affected by the tumour.

The surgeon then replaces what they’ve taken away. This may involve using plastic surgery or reconstructive surgery (see skin graft or soft tissue graft) to replace the tissue that’s been removed, or it may involve surgery to replace any bone that’s been removed. If you need to have bone removed, the surgeon replaces it with a specially designed metal implant called an endoprosthesis.

If the cancer affects a bone in or near a joint, your joint can be replaced with an artificial one.

Before the operation

Your physiotherapist might give you exercises before your operation to strengthen your muscles.

If you've had chemo, you'll need two or three weeks to recover from the treatment before you can have your operation. Doctors will check that your blood count has returned to normal. They'll also make sure that you're well enough to have the operation. If you’ve lost weight or been off your food, you might need to build yourself up a bit. The hospital dietitian can help you do this. You’ll probably also see a physiotherapist, who’ll give you exercises to do to strengthen your muscles.

If you want to have a look at the endoprosthesis that will be put in during your surgery, your nurse or surgeon can usually arrange this.

After the operation

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

You’ll probably have a tube, called a drain, coming out of your wound. This stops fluid building up in your wound. A nurse will take it out after about 3-4 days or when there isn’t very much fluid coming out of the wound.

You won’t feel like eating or drinking much for a few hours after your operation, so you’ll be given fluids into a vein.

You’ll probably feel a bit sore to begin with, but you’ll be given strong painkillers to help. It’s important to tell the nurses if you need extra pain relief. The pain usually starts to get better within 2-3 days. As time goes on you'll need fewer painkillers and will start to feel a lot more comfortable. Be honest about how you are feeling to your doctors and nurses and they can make sure that you are comfortable and as pain free as possible.

Once you’re home you’ll probably feel a bit better and stronger every day. But if you do have any problems it’s important to get them treated right away, as a delay could mean they get much more serious.

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

If you notice any new redness, soreness or swelling in the limb that’s been operated on, it’s really important to get in touch with your doctor right away. You might have an infection that needs treatment, so you’ll need to see the surgeon who did your operation without delay.

There’s more information about the long-term effects of limb-sparing surgery in our section on living with and after a soft tissue sarcoma.

Amputation

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

Before the operation

Your doctor will talk things through with you so that you fully understand what’s involved. Losing a limb can feel very scary and you might want 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 operation

You’ll have bandages or a plaster cast on the area that’s been operated on. There’ll be a drain coming from the wound to remove any excess fluid that builds up. The drain is usually taken out by a nurse on the ward after 3-4 days or when there is no fluid coming from the wound. You’ll also be given fluid into a vein in your arm until you feel like eating and drinking again.

You’ll probably feel a bit sore after your operation, especially for the first 2-4 days, but you'll be given 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’s been removed. This is known as 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 a day or so after your operation. They’ll show you exercises to keep your muscles strong and flexible.

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

Once you’re up and around you’ll 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’ll take a few months for your limb to heal to its final shape after the operation, so you won’t be fitted with your permanent prosthesis (artificial limb) until this happens. In the meantime, you’ll be given a temporary prosthesis and you’ll start learning how to use it in physiotherapy. A specialist team will help you recover from the operation and get you used to the artificial limb that you’re fitted with.

You'll be able to go home about two weeks after your operation.

You can read more about recovering after the operation in our information about living with and after a soft tissue sarcoma

Remember that it may be possible to talk to someone who has been through the same as you. Your doctor or nurse can arrange this.   

Radiotherapy

Radiotherapy treats cancer by using high-energy x-rays to destroy cancer cells while doing as little harm as possible to normal cells. There’s more information about it in the radiotherapy section.

For soft tissue sarcomas radiotherapy can be given in a number of situations:

  • 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. You’ll usually have it about 4-6 weeks after the operation, when your wound has healed
  • before surgery - to shrink the tumour and make it easier to remove with an operation
  • when it isn’t possible to remove the sarcoma with an operation

Radiotherapy is generally given every weekday (Monday-Friday). Each treatment takes a few minutes.

Side effects

Radiotherapy isn't painful, but it can cause some side effects. After a few treatments you may begin to feel tired, and your skin may go red or get darker and feel sore or itchy around the area that’s being treated. This gets better once your radiotherapy treatment is over.

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

We also have more info about:

 If you're looking for information about soft tissue sarcomas in people of all ages, see our general soft tissue sarcoma information.

Living with a soft tissue sarcoma

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

Life after limb-sparing surgery

The long-term effects of limb-sparing surgery depend on which part of your body is affected and what kind of operation you have. 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’ll be given exercises to do to help you get your limb working as well as possible. These can be hard work, but it’s important to keep going with them.

Joint replacements

If you’ve had a joint replacement, you’ll be able to walk and 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 may have for what activities you can do.

 If you’ve had a shoulder replacement you might find it harder to lift your arm above your shoulder. After any kind of joint replacement, you will be given physiotherapy. It’s very important to do the exercises your physiotherapist shows you so that 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 activities, including 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.

How will I feel?

You might feel lots of different emotions. You might be tearful, angry, fed up or depressed at times. You might think you've lost all your confidence 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. You could talk to:

  • family or friends
  • a counsellor - this can be arranged by your doctor or nurse
  • other young people in a similar situation, for example through Macmillan's online community or a support group.

Share what you've been through with Macmillan's online community, which has a group for people who are 16-24 and living with cancer. It's your space to talk freely with other people who understand what you're going through.

Things will be tough as you get used to living with an amputation, but help is available.

Other people's reactions can be hard to cope with, too. You might be worried 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.

Try to choose a couple of really close relatives or friends you can call on when things get you down. You might also find it useful to talk to someone outside your close circle, like a counsellor. Most people find it helps to talk about how they're feeling or what’s on their mind.

We also have more info about:

If you're looking for information about soft tissue sarcomas in people of all ages, see our general soft tissue sarcoma information.