Treatments for childhood Ewing sarcoma of the bone

A child diagnosed with Ewing sarcoma of the bone will have a treatment plan created for them by their healthcare team. It will be based on important information about the cancer and about the child. When deciding which treatments to offer as part of the plan, the healthcare team will consider the:

• stage of childhood bone cancer

• size of the tumour and where it is
• child's age
• child's overall health, lifestyle and activity levels

Find out more about your child's healthcare team, helping your child cope with tests and treatment and tips on managing your child's care.

Localized Ewing sarcoma of the bone in children is usually treated with chemotherapy first. It may then be treated with surgery or radiation therapy.

Chemotherapy uses anticancer (cytotoxic) drugs to destroy cancer cells. It is used first to treat the tumour. When chemotherapy is used before other treatments, it is called neoadjuvant chemotherapy. Neoadjuvant chemotherapy is given for about 12 weeks.

After chemotherapy, doctors will use MRIs, CT scans or PET-CT scans to reassess the tumour and check if it is resectable. Resectable means that the entire tumour can be removed with surgery.

If the tumour is not growing and it is resectable, it will be removed by surgery. After surgery, more chemotherapy is given and radiation therapy may or may not be given. When chemotherapy is given after surgery it is called adjuvant chemotherapy.

If the tumour is not growing but it is not resectable, it is treated with radiation therapy and more chemotherapy. The tumour may then be reassessed to see if surgery is possible.

If the tumour is still growing, a different combination of chemotherapy drugs may be given. Surgery or radiation therapy may be used to control the growth of the tumour.

Chemotherapy involves combinations of different drugs given at the same time. The most common chemotherapy combinations used for localized Ewing sarcoma of the bone are:

• vincristine, doxorubicin and cyclophosphamide (Procytox), alternating with etoposide (Vepesid) and ifosfamide (Ifex)

• doxorubicin, etoposide and ifosfamide with or without vincristine
• vincristine, doxorubicin and ifosfamide

Cyclophosphamide and ifosfamide can irritate the bladder. When these chemotherapy drugs are used, mesna (Uromitexan) is also given to protect the bladder.

Find out more about chemotherapy for childhood bone cancer.

After chemotherapy (and sometimes radiation therapy), surgery is used to remove the tumour. The goal of surgery is to remove the entire tumour. A wide resection removes the tumour and some healthy tissue around it. Doctors may use other terms for wide resection, such as wide local excision or en bloc resection. The type of surgery done will depend mostly on the size of tumour and where it is.

Tumours in the arms or legs are treated with limb-sparing surgery, amputation or rotationplasty.

• Limb-sparing surgery removes a tumour in an arm or leg but does not remove the limb. There may be several options for reconstruction available depending on your child's age, future growth and desired lifestyle.
• Amputation removes part or all of an arm or leg. It may be done when it is not possible to do limb-sparing surgery.
• Rotationplasty is a surgical procedure to treat tumours that occur near the knee.

Tumours in the ribs may be removed with surgery. The ribs can be rebuilt with artificial material.

Tumours in the pelvic (hip) bones can be hard to treat with surgery and radiation therapy may be used instead. But if the tumour responds well to chemotherapy and radiation therapy, surgery may be an option.

Tumours in the spine can be hard to treat with surgery, so radiation therapy is often used. If surgery is used, radiation therapy is usually given after surgery.

Find out more about surgery for childhood bone cancer.

Radiation therapy uses high-energy rays or particles to destroy cancer cells. It may be used to treat the tumour when surgery is not possible. Sometimes radiation therapy helps to shrink the tumour and make surgery possible. It may also be used after surgery if there are cancer cells in the tissue that is removed along with the tumour. (These are called positive margins.)

Find out more about radiation therapy for childhood bone cancer.

Metastatic Ewing sarcoma of the bone is usually treated with chemotherapy followed by surgery or radiation therapy or both.

Chemotherapy uses anticancer (cytotoxic) drugs to destroy cancer cells. It is the primary treatment for metastatic Ewing sarcoma of the bone. Chemotherapy is given for 12 to 16 weeks. After at least 12 weeks of chemotherapy, doctors will use imaging tests, such as MRIs and CT scans, to reassess the tumour. They check how well the cancer responded to treatment and plan further treatment based on that.

Chemotherapy involves combinations of different drugs given at the same time. The most common chemotherapy combinations used for metastatic Ewing sarcoma of the bone are:

• vincristine, doxorubicin and cyclophosphamide, alternating with etoposide and ifosfamide
• vincristine, doxorubicin and cyclophosphamide
• vincristine, doxorubicin and ifosfamide
• vincristine, ifosfamide, doxorubicin and etoposide

Cyclophosphamide and ifosfamide can irritate the bladder. When these chemotherapy drugs are used, mesna is also given to protect the bladder.

Find out more about chemotherapy for childhood bone cancer.

Surgery may be used to remove the primary tumour and metastases in other bones or a lung. It is done after chemotherapy (or radiation therapy, in some cases) if the cancer has spread to only a few small areas.

Find out more about surgery for childhood bone cancer.

Radiation therapy uses high-energy rays or particles to destroy cancer cells. It may be used before surgery, after surgery or alone. Radiation therapy may be given to the primary tumour as well as to areas where the cancer has spread, such as other bones or the lungs.

Radiation therapy may be given to bone metastases to relieve symptoms (called palliative therapy). It may also be given to treat lung metastases.

Chemotherapy may be given along with radiation therapy.

There are different types of radiation used. The dose of radiation depends on where it is being used.

Find out more about radiation therapy for childhood bone cancer.

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

• the size of the tumour and where it is
• whether the tumour has spread to other parts of the body
• what treatments the child has already received
• how long since the child's last treatment

Recurrent Ewing sarcoma of the bone may be treated with chemotherapy, radiation therapy, high-dose chemotherapy with stem cell rescue or a combination of these treatments.

Chemotherapy uses anticancer (cytotoxic) drugs to destroy cancer cells. Chemotherapy with one or more of the following drugs may be given, depending on what chemotherapy was used before:

• cyclophosphamide and topotecan
• temozolomide (Temodal) and irinotecan
• temozolomide, irinotecan and vincristine
• temozolomide and vincristine
• ifosfamide and etoposide
• gemcitabine and docetaxel
• ifosfamide, carboplatin and etoposide

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 (called palliative therapy). Radiation therapy may be used to treat cancer that has spread to other bones and the lungs. Find out more about radiation therapy for childhood bone cancer.

Surgery may be used to remove tumours that recur in the lungs. If radiation therapy was not used before to treat the lungs, it may be given after surgery if needed. Find out more about surgery for childhood bone cancer.

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 stem cell transplants.

Clinical trials look at new and better ways to prevent, find and treat cancer. Many children with cancer are treated in a clinical trial. Because childhood cancer is rare, clinical trials usually involve several different childhood cancer treatment centres. Being part of a trial is always voluntary.

Find out more about clinical trials and enrolling your child in a clinical trial.

Follow-up after treatment is an important part of cancer care. Children will need to have regular follow-up visits, especially in the first 10 years after treatment has finished. These visits allow the healthcare team to monitor your child's progress and recovery from treatment and to watch for recurrence. Find out more about follow-up care, life after treatment and long-term survivorship.

Treatment is successful for many children with cancer, but in some cases it isn't. When cancer is advanced, the focus of treatment changes from a cure to quality of life. There is help and support available. Find out more about when a cure is not possible.