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Chemotherapy (chemo) is the use of drugs to treat cancer. The drugs are usually given into a vein (IV) or by mouth and can reach and destroy cancer cells throughout the body.

Typically, chemo is the first treatment given (neoadjuvant chemo), followed by surgery and/or radiation therapy. In some people with Ewing sarcoma, chemo can shrink the main tumor, which might make surgery easier or the radiation field smaller. More chemo is often given again after surgery and/or radiation (known as adjuvant chemo) to kill any cancer cells that are left.

Even if the Ewing tumor is localized, the cancer would likely come back in other parts of the body if chemotherapy isn't given. Most people with Ewing sarcoma have very small areas of cancer spread (micrometastases) that can’t be seen on imaging tests. Giving chemo along with surgery and/or radiation helps lower the risk of the cancer coming back.

Chemo is given in cycles, with a period of treatment (often a few days in a row) followed by a rest period to give the body time to recover. Each cycle lasts a couple of weeks.

Which chemo drugs are used to treat Ewing sarcoma?

The drugs most often used to treat Ewing sarcoma include:

• Vincristine
• Doxorubicin
• Cyclophosphamide
• Ifosfamide
• Etoposide

In the United States, the most common chemo regimen is known as VDC/IE. It alternates between 2 combinations of drugs (vincristine/doxorubicin/cyclophosphamide and ifosfamide/etoposide), given every 2 to 3 weeks.

Most patients get chemo for 8 to 12 weeks before surgery or radiation, and more chemo afterward as well. Usually, about 14 to 17 cycles of chemo are given, which can take up to a year to complete, depending on the schedule.

Some doctors may use slightly different drug combinations. These often include many of the same chemo drugs listed above, although other drugs might be used as well.

Other chemo drugs may be used if Ewing sarcoma comes back after treatment. These include:

• Topotecan
• Cyclophosphamide
• Temozolomide
• Irinotecan
• Docetaxel
• Gemcitabine

Possible side effects of chemo

Chemo drugs affect cells that grow fast. While cancer cells grow fast, other healthy cells in the body do too – for example, blood-forming cells, hair cells, and cells that make up the lining of our gut. Side effects result from damage to fast-growing healthy cells caused by these drugs.  The specific side effects of chemo drugs depend on the type, dose, and the length of time they are given.

Common side effects of many chemo drugs include:

• Nausea and vomiting
• Loss of appetite
• Diarrhea or constipation
• Hair loss
• Mouth sores

Chemo can damage the bone marrow, where new blood cells are made. This can lead to low blood cell counts, which can result in:

• Increased chance of infection (from low white blood cells)
• Easy bruising or bleeding (from low blood platelets)
• Fatigue (from low red blood cells)

Most of these side effects tend to go away after treatment is finished. There are often ways to make these side effects less severe. For example, drugs can be given to help prevent or reduce nausea and vomiting or to help get blood counts back to normal levels. Be sure to discuss any questions you have about side effects with the cancer care team. Tell them about any side effects you are having so they can be managed.

Long-term side effects of chemo

Chemotherapy can also have some longer-term side effects. For example, some drugs can increase the risk of later developing another type of cancer (such as leukemia). While this is a serious risk, it is not common, and the small increase in risk must be weighed against the importance of chemotherapy in treating Ewing sarcoma.

Learn more in After Treatment for Ewing Sarcoma.

Side effects of certain chemo drugs

Some chemo drugs can have specific side effects. For example:

The doctors and nurses will watch closely for side effects. Do not hesitate to ask the cancer care team any questions about side effects.

For more information on possible late or long-term side effects of chemo, including infertility and second cancers, see Long-term and Late Effects of Cancer Treatment or Long-term and Late Effects of Childhood Cancer Treatment.