Rhabdomyosarcoma (RMS) is a type of soft tissue sarcoma. The information here 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 rare types of cancer that develop in the supporting or connective tissues of the body. There are two main types, soft tissue sarcomas and bone sarcomas.
Soft tissue sarcomas can develop in muscle, fat, blood vessels, or any of the other tissues that support, surround and protect the organs of the body.
Bone sarcomas can develop in any of the bones of the body, but may also develop in the soft tissue near bones.
Soft tissue sarcomas are rare. About 3,300 new cases are diagnosed each year in the UK. There are several different types of soft tissue sarcoma.
Rhabdomyosarcomas grow in the active muscles of the body. These are muscles that we have control over (for example, to move arms or legs). They are also known as skeletal or striated muscles. The most common places for rhabdomyosarcoma to be found are 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 arms and legs, 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.
Soft tissue sarcomas may occur in an area that has previously been treated with radiotherapy for another type of cancer. The sarcoma won’t usually develop until at least 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.
This test uses sound waves to 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 only 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.
We have a video about having a CT scan at macmillan.org.uk/testsandscans
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 or a pacemaker, it will not be possible for you to have this test. In this situation, another type of scan may be used.
This is a combination of a CT scan and a positron emission tomography (PET) scan. A PET scan uses low-dose radiation to measure the activity of cells in different parts of the body.
PET/CT scans give more detailed information about the part of the body being scanned. You may have to travel to a specialist centre to have one.
You can’t eat for six hours before the scan, although you may be able to drink. A mildly radioactive substance is injected into a vein, usually in your arm. The radiation dose used is very small. The scan is done after at least an hour’s wait. It usually takes 30-90 minutes. You should be able to go home after the scan.
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.
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.
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 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, are likely to grow more quickly, and are more likely to spread. Embryonal and alveolar types of rhabdomyosarcoma are always high-grade.
Staging of rhabdomyosarcoma
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The stage of a cancer is a term used to describe its size and whether it has spread beyond its original site. Knowing the particular type and stage of a cancer helps the doctors plan the most appropriate treatment.
Most people are grouped depending on whether cancer is found in only one part of the body (localised disease) or whether the cancer has spread from one part of the body to another (metastatic disease).
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.
Treatment for rhabdomyosarcoma
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As sarcomas are rare, they are usually treated by a team of doctors and other health care professionals at a specialist hospital. This means that you may have to travel some distance to have your treatment.
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 decide on the best form of treatment for you. They will then discuss this with you.
Knowing the particular type of rhabdomyosarcoma, its stage and whether it can be completely removed during an operation helps the doctors put people into three risk groups. Knowing which risk group you are in helps doctors to plan the best treatment for you. Your doctor or nurse can give you more information about risk groups.
Chemotherapy for rhabdomyosarcoma
Treatment often begins with chemotherapy 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 get rid of any cancer cells that have not been removed, or which may have spread to other parts of the body.
Surgery for rhabdomyosarcoma
Surgery is carried out if the tumour is small. If it's possible to remove it completely, surgery may be used on its own.
Radiotherapy for rhabdomyosarcoma
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.
Radiotherapy may be given as an alternative to surgery, when it’s not possible to remove the tumour. Sometimes chemotherapy is given at the same time as radiotherapy (chemoradiation).
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 still receive the best standard treatment available.
Follow-up after rhabdomyosarcoma treatment
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After your treatment is completed, you will have regular check-ups and x-rays. Your specialist will advise you on how frequently you need to be seen. Follow-up will continue for several years. If you have any problems, or notice any new symptoms in between your regular appointments, let your doctor know as soon as possible.
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 receive 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 specially 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 outside 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 and 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.
Cancer52 is an alliance of more than 50 organisations working to address the inequalities that exist in policy, services and research into the less common cancers and to improve outcomes for people with these highly challenging diseases.
Rarer Cancers Foundation
Rare Cancers Foundation is a UK charity that offers general advice and information about rare and less common cancers, facilitates supportive networking between patients and carers, and works to improve services for people with rarer cancers.
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.
Teenage Cancer Trust
Teenage Cancer Trust is a national charity providing specialist units, support, education and information for teenagers with cancer and their families.
This information has been compiled using a number of reliable sources, including:
DeVita V, et al. DeVita, Hellman, and Rosenberg's Cancer: Principles & Practice of Oncology (9th edition). 2011. Lippincott Williams & Wilkins. Philadelphia.
National Institute for Health and Clinical Excellence (NICE). Improving outcomes for people with sarcoma. March 2006.
Grimer R, et al. UK Guidelines for the Management of Soft Tissue Sarcomas. British Sarcoma Group. 2010.
Raghavan D, et al. Textbook of Uncommon Cancer (3rd edition). 2006. Wiley.
National Cancer Intelligence Network (NCIN). Bone and Soft Tissue Sarcomas: UK Incidence and Survival 1996 to 2010. November 2013.
Weiss, et al. Enzinger and Weiss's Soft Tissue Tumors (5th edition). 2008. Mosby.
Thank you to Dr Aisha Miah, Consultant Clinical Oncologist; Professor Ian Judson, Professor of Cancer Pharmacology; and the people affected by cancer who reviewed this information.
Reviewing is just one of the ways you could help when you join our Cancer Voices network.