Rhabdomyosarcomas belong to a group of cancers called soft tissue sarcomas. This section should ideally be read with our general information about soft tissue sarcomas.
We hope this information answers your questions. If you have any further questions, you can ask your doctor or nurse at the hospital where you are having your treatment.
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. Around 1 out of 100 (1%) of all cancers are soft tissue sarcomas.
There are two main types of muscles in the body:
active muscles that we control (eg to move arms or legs)
involuntary muscles that we have no control over (eg in the stomach or heart).
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 of the body.
Very rarely, rhabdomyosarcoma can also be found in other places, such as in the prostate gland, middle ear, and bile duct system.
Types of rhabdomyosarcoma
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There are three subtypes of rhabdomyosarcoma. They tend to affect different age groups.
This type is most common in young children, usually under the age of six. It often appears in the head and neck region, and especially in the tissues around the eye (orbital rhabdomyosarcoma). Another common area for these tumours to develop 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. This type of 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. A cure is now usually possible with this course of treatment.
This type tends to occur in older children and adolescents. It occurs more often in the limbs, or in the chest or abdomen. It is usually treated with chemotherapy, along with surgery or radiotherapy.
This type tends to occur in middle-aged people. It is usually treated with a combination of chemotherapy, surgery and radiotherapy. Unlike rhabdomyosarcomas in children, treatment is often not as effective.
For unknown reasons, slightly more men are diagnosed with rhabdomyosarcoma than women.
Causes of rhabdomyosarcomas
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The exact causes of rhabdomyosarcomas are unknown. Researchers are trying to 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 won’t usually develop until about 10 years after the radiotherapy treatment.
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, and some types of herbicides (weed killers) and dioxins.
Signs and symptoms of rhabdomyosarcoma
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Most rhabdomyosarcomas are diagnosed after a person develops symptoms. These may include:
a lump that can be seen or felt - it may or may not be painful
bleeding from the nose, vagina, rectum or throat, if the tumour is in these areas
tingling, numbness, pain and loss of movement, if the tumour presses on nerves in the area
protrusion of the eye, where the eye seems to be pushed forward
a drooping eyelid due to a tumour behind the eye.
If you notice any of these symptoms, you should contact your GP. Remember that these symptoms can also be caused by conditions other than cancer.
How rhabdomyosarcoma is diagnosed
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Usually you begin by seeing your family doctor (GP), who will examine you. You will be referred to a hospital specialist for any necessary tests, 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 on the part of the body being investigated. If you’re having other tests that aren’t listed here, our cancer support specialists can give you further information.
This uses x-rays to take a picture of your chest, to check that your lungs and heart are healthy.
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 only drink clear fluids (nothing fizzy or milky) for 4-6 hours before the scan.
Once you’re lying comfortably on your back, a gel is spread on your abdomen. A small device like a microphone (called a probe) 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 build up a three-dimensional picture of the inside of the body. The scan is painless and takes 10-30 minutes. CT scans use small amounts of radiation, which is very unlikely to harm you or 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. This may make you feel hot all over for a few minutes. If you’re allergic to iodine or have asthma, you could have a more serious reaction to the injection, so it's important to let your doctor know beforehand.
You'll probably be able to go home as soon as the 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. You'll be able to hear, and speak to, the person operating 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.
The results of the previous tests may make your doctor strongly suspect that you have cancer. The only way to be sure is to take 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 needle is passed into the tumour through the skin. A CT or ultrasound scan may be used at the same time, to make sure that the biopsy is taken from the right place. A piece of tissue is then removed.
When the tissue is looked at under a microscope, the pathologist will be able to tell if it is cancerous (malignant) or not cancerous (benign). 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.
Staging and grading of rhabdomyosarcoma
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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 decide on the most appropriate treatment.
Generally, sarcomas are divided into four stages:
stage 1 – the sarcoma is small and localised
stages 2 or 3 – it has spread into surrounding structures
stage 4 – it has spread into other parts of the body.
If the cancer has spread to distant parts of the body, it is known as secondary (or metastatic) cancer.
Grading of rhabdomyosarcoma
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Grading refers to the appearance of cancer cells under a microscope. The grade gives an idea of how quickly cancer may develop. Grading of soft tissue sarcomas can sometimes be difficult, especially for the less common types.
Low-grade means the cancer cells look very much 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. Embryonal and alveolar types of rhabdomyosarcoma are always high-grade.
Treatment for rhabdomyosarcoma
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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 decide on the best form of treatment for you.
Children and young adults are generally treated with chemotherapy first to shrink the tumour. Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy the cancer cells. The tumour is then removed with surgery. If it has disappeared following chemotherapy, then radiotherapy can be used. Radiotherapy uses high-energy rays to treat cancer.
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's 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 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.
Clinical trials for rhabdomyosarcoma
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Research into treatments for rhabdomyosarcoma is ongoing and advances are being made. Cancer doctors use clinical trials to assess new treatments.
Before any trial is allowed to take place, it must be approved by an ethics committee, which protects the interests of the patients taking part.
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 it causes. Your specialist will inform you of any potential side effects and how to deal with them before you agree to treatment.
You may experience many different emotions. Anger, guilt, anxiety and fear are some of the most common feelings 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 can be difficult and distressing to admit you have them.
You don't 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 member of their religious faith. We 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.
Sarcoma UK provides information and support to anyone affected by sarcoma, and aims to achieve the best possible standard of treatment and care for people with sarcoma.
Rarer Cancers Foundation
Rare Cancers Foundation offers advice and information to people with rare and less common cancers. Tries to put people in touch with others affected by a similar cancer for support and to share experiences.
This information has been compiled using a number of reliable sources, including:
Improving outcomes for people with sarcoma. March 2006. National Institute for Health and Clinical Excellence (NICE).
Raghavan, et al. Textbook of Uncommon Cancers. 3rd edition. 2006. Wiley.
Weiss, et al. Enzinger and Weiss's Soft Tissue Tumors. 5th edition. 2008. Mosby.
With thanks to: Professor Ian Judson, Professor of Cancer Pharmacology; and the people affected by cancer who reviewed this edition.
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