Rhabdomyosarcoma

Rhabdomyosarcoma is a type of soft tissue sarcoma. It develops from skeletal (striated) muscle cells. This is the type of muscle that you can control (voluntary muscle). A soft tissue sarcoma is a type of cancer.

Rhabdomyosarcomas are more common in children and adolescents, and rare in adults.

There are 3 distinct types of rhabdomyosarcoma. These are:

• embryonal rhabdomyosarcoma
• alveolar rhabdomyosarcoma 
• pleomorphic rhabdomyosarcoma

Knowing the type of rhabdomyosarcoma helps your specialist decide on the best treatment for you. 

Treatment overview

The type of treatment you might have depends on the level of risk of your cancer. Your doctor will look at several factors to decide what the risk of your rhabdomyosarcoma is. These factors include:

• the stage of your rhabdomyosarcoma
• your age
• the size of the tumour
• the type of rhabdomyosarcoma 
• whether you have a gene  made up by joining parts of two different genes (fusion gene)
• where the tumour is in your body
• whether the cancer has spread to any lymph nodes

Depending on the level of risk, you will have surgery. Then you may have chemotherapy on its own. Or chemotherapy alongside radiotherapy. 

Surgery

You are likely to have surgery if it is possible to remove the sarcoma. The surgeon removes the cancer along with a border of healthy looking tissue around it. After the operation, they send the tumour to the laboratory. A pathologist examines the border of healthy tissue for cancer cells.

Chemotherapy 

The chemotherapy drugs most commonly used are:

• vincristine
• actinomycin D
• cyclophosphamide
• ifosfamide

When surgery is not possible 

If surgery is not possible you might have radiotherapy to the sarcoma. Sometimes surgery to remove rhabdomyosarcoma may have a large effect on the way you look. In situations where surgery would be very disfiguring, your doctor may offer you radiotherapy instead of surgery. 

Types and treatment

Embryonal rhabdomyosarcoma

Embryonal rhabdomyosarcoma is more common in children. It usually occurs in the head and neck region, the bladder or genital area. 

Your treatment depends on where in the body the rhabdomyosarcoma is. Surgery is usually part of the treatment. Chemotherapy tends to work well with this type of sarcoma. You might have chemotherapy before surgery to shrink the cancer. This makes it easier to remove. This is called neoadjuvant treatment. 

Chemotherapy after surgery aims to help stop the cancer from coming back. This is called adjuvant treatment. 

If you can't have surgery 

Some people might not be able to have surgery. This is because the sarcoma is in a place where it is not possible to completely remove it. For example, it might be behind the nose or in the eye socket. In this situation, you might have a combination of radiotherapy and chemotherapy.

Alveolar rhabdomyosarcoma

This occurs in the arms or legs of older children and young people but can also occur in the muscles of the trunk.

Alveolar rhabdomyosarcoma is usually treated with surgery. Then you have radiotherapy to the area where the sarcoma was.

Radiotherapy aims to reduce the chance of the tumour coming back in the same place. Chemotherapy is usually given before or after surgery.

Pleomorphic rhabdomyosarcoma

This type of rhabdomyosarcoma usually occurs in adults, in the arms or legs.

The main treatment is surgery. After surgery you usually have radiotherapy alongside chemotherapy. This aims to lower the risk of sarcoma coming back. 

Chemotherapy does not work as well with pleomorphic rhabdomyosarcoma compared to other types of rhabdomyosarcoma.

Coping

Coping with a diagnosis of a rare cancer can be especially difficult. Knowing more about your sarcoma and its treatment can make it easier. It can help you to make decisions and cope with what happens.

Sarcoma UK has support and information for people affected by soft tissue and bone sarcoma.

The Rare Cancer Alliance offer support and information to people with rare cancers.

Talking to other people who have the same thing can also help.

Our discussion forum Cancer Chat is a place for anyone affected by cancer. You can share experiences, stories and information with other people who know what you are going through.