Treatments for Ewing sarcoma

Last medical review:

The following are treatment options for Ewing sarcoma. Your healthcare team will suggest treatments based on your needs and work with you to make a treatment plan.

Surgery

Surgery is the standard treatment for Ewing sarcoma. The following types of surgery may be used.

Wide resection

Wide resection removes the bone tumour and a small amount of tissue and bone around it (called a surgical margin). Doctors may use other terms for wide resection, such as en bloc resection or wide excision.

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

Reconstructive surgery

Reconstructive surgery is done at the same time as surgery to remove the tumour. Bone and tissue grafts may be used to replace tissues removed during surgery. A joint removed with surgery may be replaced with a metallic artificial joint (endoprosthesis).

Amputation

Amputation removes all or part of the arm or leg with the tumour. It may be done if a Ewing sarcoma has grown into the nerves or blood vessels or if the tumour is very big. Amputation may also be done if the cancer comes back in the same area after limb-sparing surgery. Most people who have an amputation will use an artificial limb (prosthesis) after surgery.

Surgery for lung metastases

If bone cancer spreads to the lungs, it is called lung metastasis. Surgery may be used to treat Ewing sarcoma that has spread to the lungs if doctors think that all of the tumour can be removed.

Doctors will often use a wedge resection to remove the tumour along with a triangle shaped piece of the lung around it. It’s often done at the same time as surgery to remove the primary bone tumour.

Surgery may be done again to remove lung tumours that come back, depending on the size of the tumours and where they are.

Find out more about surgery for bone cancer.

Chemotherapy

Chemotherapy uses drugs to destroy cancer cells. It is almost always offered for Ewing sarcoma.

Chemotherapy is commonly used to shrink the tumour before surgery (called neoadjuvant chemotherapy). It is also given to destroy cancer cells left behind after surgery and reduce the risk that the cancer will come back (called adjuvant chemotherapy).

The most common chemotherapy drug combinations used to treat Ewing sarcoma are:

  • VDC/IE – vincristine, doxorubicin and cyclophosphamide, alternated with ifosfamide and etoposide
  • VIDE – vincristine, ifosfamide, doxorubicin and etoposide
  • VAI – vincristine, dactinomycin and ifosfamide

If a Ewing sarcoma does not respond to drugs used in earlier treatments or if it comes back, the following drug combinations may be used:

  • irinotecan and temozolomide, sometimes with vincristine
  • topotecan and cyclophosphamide
  • gemcitabine and docetaxel

Find out more about chemotherapy for bone cancer.

Radiation therapy

Radiation therapy uses high-energy rays or particles to destroy cancer cells. It is sometimes offered for Ewing sarcoma.

External radiation therapy may be used to:

  • treat Ewing sarcoma if surgery can’t be done
  • destroy cancer cells if the tumour couldn’t be completely removed during surgery
  • treat lung metastases
  • relieve pain and control symptoms of cancer that has spread (metastasized) or come back (recurred) (called palliative therapy)

Find out more about radiation therapy for bone cancer.

Clinical trials

Talk to your doctor about clinical trials open to people with bone cancer in Canada. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.

Expert review and references

  • Michael Monument, MD, MSc, FRCSC
  • American Cancer Society. Ewing Family of Tumors. 2021. https://www.cancer.org/.
  • Strauss SJ, Frezza AM, Abecassis N, et al. Bone sarcomas: ESMO–EURACAN–GENTURIS–ERN PaedCan Clincial Practice Guideline for diagnosis, treatment and follow-up. Annals of Oncology. 2021: 32(12):1520–1536.
  • O'Donnell RJ, DuBrois SG, Haas-Kogan DA, Braunstein SE, Hameed M. Sarcomas of Bone. DeVita VT Jr, Lawrence TS, Rosenberg S. eds. DeVita Hellman and Rosenberg's Cancer: Principles and Practice of Oncology. 12th ed. Philadelphia, PA: Wolters Kluwer; 2023: Kindle version, chapter 61, https://read.amazon.ca/?asin=B0BG3DPT4Q&language=en-CA.
  • National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Bone Cancer Version 2.2024. 2024.
  • Samuel LC. Bone and soft tissue sarcomas. Yarbro CH, Wujcik D, Gobel B (eds.). Cancer Nursing: Principles and Practice. 8th ed. Burlington, MA: Jone & Bartlett Learning; 2018: Kindle version, chapter 46, https://read.amazon.ca/?asin=B01M6ZZEWT&ref_=kwl_kr_iv_rec_1.

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