All children with rhabdomyosarcoma will probably be treated with chemotherapy at some point. Even if it is thought that the cancer was completely removed by surgery, without chemotherapy it is likely to come back.

Chemotherapy is the use of drugs to treat cancer. Chemotherapy is systemic therapy, meaning that the drug enters the bloodstream and reaches throughout the body to destroy cancer cells. This makes chemotherapy useful for killing rhabdomyosarcoma cells that have spread to the lymph nodes, bone marrow, liver, lungs, or other organs.

Following surgery, any tiny deposits of rhabdomyosarcoma that remain can often be destroyed by chemotherapy. If larger areas of tumor remain after surgery, chemotherapy (along with radiation) can often shrink these areas. In some cases this may allow further surgery to completely remove the remaining tumor.

Drugs used to treat rhabdomyosarcoma

There are many kinds of chemotherapy drugs. Some of them can be taken by mouth, but most are injected into a vein. The drugs used depend to some extent on which risk group the child is in (described in the section "How is rhabdomyosarcoma staged?")

The main drugs used to treat children in the low-risk group are vincristine and dactinomycin (also known as actinomycin-D). This combination is often referred to as VA. In some cases cyclophosphamide may be added as well. This 3-drug combination is referred to as VAC.

The VAC regimen is the most common combination used for the intermediate-risk group. Irinotecan or topotecan may be added as well. Other drugs used to treat rhabdomyosarcoma include ifosfamide, etoposide, and doxorubicin.

The same drugs are also used for children in the high-risk group (those with metastatic disease), although they have not been shown to be as successful. New drugs and drug combinations are continually being studied by the Soft Tissue Sarcoma Committee of the Children's Oncology Group and other research groups. It is hoped that they will improve the survival rate in the high-risk group.

Possible side effects

Chemotherapy drugs work by attacking cells that are dividing quickly, which is why they often work against cancer cells. But other cells in the body, such as those in the bone marrow, the lining of the mouth and intestines, and the hair follicles, also divide quickly. These cells are also likely to be affected by chemotherapy, which can lead to side effects.

The side effects of chemotherapy depend on the type of drugs, the doses, and how long they are taken. Possible side effects can include:

• hair loss
• mouth sores
• loss of appetite
• nausea and vomiting
• increased chance of infections (from low white blood cell counts)
• easy bruising or bleeding (from low blood platelet counts)
• fatigue (from low red blood cell counts)

These side effects are usually short-term and go away once treatment is finished. Your child's doctor and treating team will watch closely for any side effects that develop. There are often ways to lessen these side effects. For example, drugs can be given to help prevent or reduce nausea and vomiting. Be sure to ask your doctor or nurse about medicines to help reduce side effects, and report any side effects your child has so they can be managed effectively.

Along with the risks above, some chemotherapy drugs have specific side effects (although these are relatively uncommon). For example, ifosfamide and cyclophosphamide may damage the bladder or kidneys, which can lead to blood in the urine and other problems. Other medicines can be given to help prevent these problems.

Recent studies have shown that children under the age of 3 years are more likely to have liver damage from the chemotherapy. Doctors now use lower and very specific doses for any child younger than 3 years old.

Chemotherapy may also increase the risk of developing a second type of cancer, usually a form of leukemia, years after the rhabdomyosarcoma is cured. However, the importance of chemotherapy in treating rhabdomyosarcoma far outweighs this risk.

For more extensive information on chemotherapy, see the separate American Cancer Society document, Understanding Chemotherapy: a Guide for Patients and Families.

Last Medical Review: 09/08/2009
Last Revised: 09/08/2009