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This information is about a specific type of soft tissue sarcoma known as rhabdomyosarcoma (pronounced rab-doe-my-o-sar-coma). It should ideally be read with our general information about soft tissue sarcomas.
Rhabdomyosarcomas belong to a group of cancers called soft tissue sarcomas. Sarcomas are cancers that develop in the supporting or connective tissues of the body (such as muscle, fat, nerves, blood vessels, bone and cartilage). Soft tissue sarcomas are rare. Approximately 1,200 – 2,000 people will be diagnosed with a sarcoma each year in the UK.
There are two main types of muscles in the body:
Rhabdomyosarcomas grow in the active muscles of the body. These muscles are also known as skeletal or striated muscle. The most common places for rhabdomyosarcoma to be found include the head, neck, bladder, vagina, arms, legs and trunk.
Very rarely, rhabdomyosarcoma can also be found in other places, such as in the prostate gland, middle ear and bile duct system.
There are three different subtypes of rhabdomyosarcoma. They tend to affect different age groups.
Embryonal rhabdomyosarcoma is commonest in young children, usually under the age of six. It often appears in the head and neck region, frequently affecting the tissues around the eye (orbital rhabdomyosarcoma). Another common area for these tumours is the genitourinary region, where they can occur in the womb, vagina, bladder or prostate gland.
One type of embryonal rhabdomyosarcoma is called sarcoma botryoides. The tumour looks like a tiny mass of grapes sticking out from the nose, eye or vagina. Although the appearance of these tumours, especially in young children, can be very frightening and distressing, they usually respond very well to treatment.
Embryonal rhabdomyosarcomas are very likely to spread to surrounding tissues and to other parts of the body. They are usually treated with a combination of chemotherapy, surgery and radiotherapy. With this treatment, a cure is now usually possible.
Alveolar rhabdomyosarcoma tends to occur in older children and adolescents. They occur more often in the limbs or in the chest or abdomen. Once again, treatment is usually chemotherapy, supported by surgery or radiotherapy.
Pleomorphic rhabdomyosarcoma tends to occur in middle-aged people. The treatment is usually a combination of chemotherapy, surgery and radiotherapy. Unlike rhabdomyosarcomas in children, however, treatment is often not as effective.
For unknown reasons, slightly more males are diagnosed with rhabdomyosarcoma than females.
The exact causes of rhabdomyosarcomas are unknown, and research is ongoing to try and find out as much as possible about them.
Rhabdomyosarcomas are slightly more common in children with Li-Fraumeni syndrome, which is a genetic disorder.
Very rarely, soft tissue sarcomas may also occur in an area that has previously been treated with radiotherapy for another type of cancer. The sarcoma will not usually develop for at least ten years after the radiotherapy treatment. Improvements in targeting radiotherapy mean that the risk of developing a sarcoma after treatment is now very small.
Exposure to some types of chemicals may be linked to the development of some sarcomas. The chemicals include vinyl chloride, which is used for making plastics, some types of herbicides (weedkillers), and dioxins.
Most rhabdomyosarcomas are diagnosed after a person develops symptoms. These may include:
If you notice any of the above, contact your GP, but remember that these symptoms can also be caused by other conditions.
Usually you begin by seeing your family doctor (GP), who will examine you. You will be referred to a hospital specialist for any tests that may be necessary and for expert advice and treatment. The doctor at the hospital will take your full medical history, do a physical examination and take blood and urine samples to check your general health.
The tests you have will depend upon the part of the body that is being investigated. If you are having other tests that are not listed, our nurses can give you further information.
Chest x-ray A simple picture taken of your chest to check that your lungs and heart are healthy.
Ultrasound scan Sound waves make up a picture of the abdomen and surrounding organs. It is done in the hospital's scanning department. You will be asked not to eat, and to drink clear fluids only (nothing fizzy or milky) for 4–6 hours before the scan. Once you are lying comfortably on your back a gel is spread on your abdomen. A small device like a microphone is then rubbed over the area. The sound waves are converted into a picture using a computer. The test is completely painless and takes 15–20 minutes.
CT (computerised tomography) scan A CT scan takes a series of x-rays that builds up a three-dimensional picture of the inside of the body. The scan is painless and takes from 10 to 30 minutes. CT scans use a small amount of radiation, which is very unlikely to harm you and will not harm anyone you come into contact with. You will be asked not to eat or drink for at least four hours before the scan.
You may be given a drink or an injection of dye that allows particular areas to be seen more clearly. For a few minutes this may make you feel hot all over. 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 beforehand.
Most people are able to go home as soon as their scan is over.
MRI (magnetic resonance imaging) scan This test is similar to a CT scan, but uses magnetism instead of x-rays to build up cross-sectional pictures of your body. During the test you will be asked to lie very still on a couch inside a large metal cylinder that is open at both ends. The whole test may take up to an hour. It can be slightly uncomfortable and some people feel a bit claustrophobic during the scan. It is also very noisy and you will be given earplugs or headphones to wear. A two-way intercom allows you to talk with the people controlling the scanner.
If you have any metal implants (such as certain types of surgical clips, pacemakers, or metal in the eye from previous accidents or trauma) it will not be possible for you to have this test.
Biopsy The results of the previous tests may make your doctor strongly suspect a diagnosis of cancer. The only way to be sure is to take some cells or a small piece of tissue from the affected area to look at under a microscope. This is called a biopsy. The area is numbed using a local anaesthetic injection and then a fine needle is passed into the tumour through the skin. CT or ultrasound may be used at the same time, to make sure that the biopsy is taken from the right place.
When the cells are looked at under a microscope, the pathologist will be able to tell if they are not cancerous (benign) or cancerous (malignant). If a sarcoma is diagnosed, further tests may be done on the sample to try to find out exactly what type of sarcoma it is.
The stage of a cancer describes its size and whether it has spread beyond its original site. Knowing the particular type, grade and stage of the cancer helps the doctors to decide on the most appropriate treatment.
Generally sarcomas are divided into four stages, from stage 1: small and localised; to stages 2 or 3: spread into surrounding structures; to stage 4: spread into other parts of the body. If the cancer has spread to distant parts of the body this is known as secondary cancer (or metastatic cancer).
Grading refers to the appearance of the cancer cells under the microscope. The grade gives an idea of how quickly the cancer may develop. Grading of soft tissue sarcomas can sometimes be difficult, especially for the less common types. 'Low-grade' means that the cancer cells look very like the normal cells of the soft tissues. 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.
The treatment for rhabdomyosarcoma depends on a number of things, including your general health, and the size and position of the tumour. The results of your tests will help your doctor to decide on the best form of treatment for you.
Children and young adults are generally treated first with chemotherapy to shrink the tumour. The tumour is then removed with surgery.
Sometimes chemotherapy is given after surgery, to try to destroy any cancer cells that have not been removed or which may have spread to other parts of the body.
Surgery is carried out if the tumour is small. If it is possible to remove it completely, surgery may be used on its own.
For larger sarcomas, and where there is a possibility of cancer cells being left behind, radiotherapy is usually used as well as surgery. Radiotherapy may be given before the operation to shrink the tumour and make it easier to remove. It may be used after the operation to try to destroy any tiny cancer cells that may not have been removed.
Treatment for young children has to be carefully planned to minimise the effect of the treatment on their growth and development.
The treatment of soft tissue sarcomas is discussed in more detail in our general information about soft tissue sarcomas.
Research into treatments for rhabdomyosarcoma is ongoing and advances are being made. Cancer doctors use clinical trials to assess new treatments.
You may be asked to take part in a clinical trial. If you decide to take part in a trial, your doctor must discuss the treatment with you so that you have a full understanding of the trial and what it means to take part. You may decide not to take part or to withdraw from a trial at any stage. You will then receive the best standard treatment available.
The need for practical and emotional support will vary from person to person and may depend on the treatment you receive and any side effects the treatment may cause. Your specialist will inform you of any potential side effects and how to deal with them before you agree to treatment.
Many different emotions may affect you. Anger, guilt, anxiety and fear are some of the most common feelings that people have. You may find yourself tearful, restless and unable to sleep. You may have feelings of hopelessness and depression. These are all normal reactions, but it is often difficult and distressing to admit to them.
You do not have to cope with these feelings on your own and there are people available to help you. Some hospitals have their own emotional support services with specially-trained staff, and some of the nurses on the ward will have received training in counselling. Some people feel more comfortable talking to a counsellor outside the hospital environment or to a religious leader. Cancerbackup can put you in touch with counselling services in your area.
You may also find it helpful to talk to your family and friends about how you feel.
This section has been compiled using information from a number of reliable sources, including:
For further references, please see the general bibliography.
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