Treatments for childhood Ewing sarcoma

The following are treatment options for childhood Ewing sarcoma. The healthcare team will suggest treatments based on your child’s needs and work with you to develop a treatment plan.

Treatment for childhood Ewing sarcoma depends on:

• whether the cancer has spread

• the size of the tumour and where it is
• your child’s age
• your child’s overall health
• your child’s lifestyle and what they prefer or want

In localized childhood Ewing sarcoma, the cancer is only in the bone it started in or in nearby tissues. It’s usually treated with chemotherapy, followed by surgery or radiation therapy or both, followed by more chemotherapy.

Chemotherapy uses drugs to destroy cancer cells. It’s often given as the first treatment for localized Ewing sarcoma (called neoadjuvant chemotherapy). Your child will have an MRI or CT scan before and after chemotherapy to assess the tumour. This helps to decide on further treatment and to plan for surgery.

The most common chemotherapy combination used for localized Ewing sarcoma is vincristine, doxorubicin and cyclophosphamide, alternating with a combination of ifosfamide and etoposide.

Other chemotherapy combinations that may be used include:

• doxorubicin, etoposide and ifosfamide (sometimes including vincristine)
• vincristine, doxorubicin and ifosfamide

If ifosfamide or cyclophosphamide is used, then mesna is given at the same time. Mesna is a supportive drug used to protect the bladder from damage and lower the risk of urinary tract problems caused by these chemotherapy drugs.

If doxorubicin is used, then dexrazoxane is given at the same time. Dexrazoxane is used to protect the heart from damage caused by this chemotherapy drug.

Find out more about chemotherapy for childhood bone cancer.

Surgery may be offered for localized Ewing sarcoma in children. It’s usually offered after chemotherapy.

The main goal of surgery is to remove the whole tumour along with some normal tissue around it (called the surgical margin). The other tissues or structures that need to be removed will depend on the size of the tumour and where it is.

The following types of surgery may be offered for localized Ewing sarcoma.

Wide resection removes the bone tumour and a small amount of bone and tissue around it. Doctors may use other terms for wide resection, such as en bloc resection or wide excision. It may be used for Ewing sarcoma in the ribs, pelvis (hip), spine, arm or leg.

Limb-sparing surgery is often used to remove tumours in an arm or leg. During limb-sparing surgery, surgeons do a wide resection to remove the tumour without amputating the limb.

Rotationplasty is a type of surgery that can be done to remove a tumour near the knee. During rotationplasty, the tumour and knee joint are removed and the remaining ankle joint is rotated to function as a knee joint. Find out more about rotationplasty.

Amputation removes part or all of an arm or leg with the tumour. It may be done when it isn’t possible to do limb-sparing surgery.

Find out more about surgery for childhood bone cancer.

Radiation therapy uses high-energy rays or particles to destroy cancer cells. It’s sometimes used to treat localized Ewing sarcoma.

It may be used on its own if surgery can’t be done. Sometimes it can be used to shrink the tumour and make surgery possible. It may also be used after surgery to destroy cancer cells if the tumour couldn’t be completely removed during surgery.

Find out more about radiation therapy for childhood bone cancer.

Metastatic Ewing sarcoma is cancer that has spread from the bone to other parts of the body. It’s usually treated with chemotherapy, followed by surgery or radiation therapy or both, followed by more chemotherapy.

Chemotherapy uses drugs to destroy cancer cells. It’s the standard treatment for metastatic Ewing sarcoma.

The most common chemotherapy drugs used for metastatic Ewing sarcoma are:

• vincristine
• doxorubicin
• cyclophosphamide
• ifosfamide
• etoposide

Find out more about chemotherapy for childhood bone cancer.

Radiation therapy uses high-energy rays or particles to destroy cancer cells. It is sometimes used to treat metastatic Ewing sarcoma.

Radiation therapy may be used on its own, or it may be used before or after other treatments. It can also be used to treat bone and lung metastases. Chemotherapy may be given along with radiation therapy (called chemoradiation).

Find out more about radiation therapy for childhood bone cancer.

Your child may be offered surgery for metastatic Ewing sarcoma. Surgery may be used to remove the primary tumour. It may also be used to remove cancer that has spread (metastasized) to other bones or to a lung.

Find out more about surgery for childhood bone cancer.

Recurrent childhood Ewing sarcoma means that the cancer has come back after it has been treated.

The treatments offered for recurrent childhood Ewing sarcoma are based on:

• how long it has been since the last treatment
• the size of the tumour and where it is
• whether or not the cancer has spread to other parts of the body
• which treatments were used before

Your child may be offered the following treatments for recurrent Ewing sarcoma. The healthcare team will suggest treatments based on your child’s needs and work with you to develop a treatment plan.

Chemotherapy uses drugs to destroy cancer cells.

One or more of the following chemotherapy drugs may be given, depending on which type of chemotherapy drug was used before:

• irinotecan
• temozolomide
• vincristine
• topotecan
• cyclophosphamide
• ifosfamide
• gemcitabine
• docetaxel
• etoposide
• carboplatin

High-dose chemotherapy with stem cell rescue may be used in some cases to treat recurrent childhood Ewing sarcoma as part of a clinical trial.

Find out more about chemotherapy for childhood bone cancer.

Radiation therapy uses high-energy rays or particles to destroy cancer cells. It may be given to relieve symptoms of recurrent childhood Ewing sarcoma. Radiation therapy may also be used to treat cancer that has spread to other bones and to the lungs.

Find out more about radiation therapy for childhood bone cancer.

Your child may be offered surgery to treat recurrent Ewing sarcoma. Surgery may be used to remove tumours that recur in the lungs. Find out more about surgery for childhood bone cancer.

Targeted therapy uses drugs to target specific molecules (such as proteins) on cancer cells or inside them to stop the growth and spread of cancer. Your child may be offered targeted therapy as part of a clinical trial. Some targeted therapy drugs that may be used include:

• regorafenib

• cabozantinib

Find out more about targeted therapy.

Children with cancer may be treated in a clinical trial. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.

Treatment is successful for many children with cancer, but in some cases it isn’t. Advanced cancer is cancer that is unlikely to be cured. When cancer is advanced, the focus of treatment can change from curing the cancer to ensuring a good quality of life. There is help and support available. Find out more about when a cure is not possible.