(Peripheral Primitive Neuroectodermal Tumors [PNET]; Ewing Family of Tumors)
Pronounced: YOO-ingz sar-KOH-muh
by Diana Kohnle and Patricia Griffin Kellicker, BSN
Ewing sarcoma is a cancerous bone tumor that may occur in any bone in the body. When it occurs in soft tissue, it is called extraosseous sarcoma. The most commonly affected areas include the pelvis, thigh, lower leg, upper arm, and chest wall.
The causes of Ewing sarcoma are not fully understood. Chromosomal rearrangement that alters the genes may be a factor.
Risk Factors TOP
Because the causes of the cancer are unknown, risk factors are also not fully understood. Ewing sarcoma appears to be more common in Caucasians, teenagers, and males.
You will be asked about your symptoms and medical history. A physical exam will be done. You will be treated by a team of doctors which may include an oncologist who specializes in cancer, an orthopaedic surgeon who operates on bones, and a radiation oncologist who works with radiation to kill cancer cells.
Your bodily structures may need to be viewed. This can be done with:
Your bodily fluids and tissues may need to be tested. This can be done with:
Treatment involves a combination of chemotherapy, surgery, and radiation. Typically, patients are first treated with chemotherapy for 8-12 weeks, followed by a CT or MRI scan to evaluate the tumor. Depending on the tumor size and location, either surgery or radiation therapy follows. Chemotherapy then resumes for several months after surgery or radiation therapy.
Chemotherapy drugs are used to kill tumor cells. Your doctor will prescribe a combination of drugs to be given over a set time. This usually means every 2-4 weeks for several months.
Surgery may be used to remove the tumor, as well as rebuild the affected bone. Depending on the location of the tumor, a bone graft or prosthesis may be needed. There are special types of prostheses that expand as the bone grows. Sometimes, several surgeries are needed to make sure the limb functions properly.
Radiation Therapy TOP
Radiation therapy uses high-energy x-rays to kill tumor cells. It may be used with surgery or instead of surgery. It is usually given over several weeks and is followed by chemotherapy.
There are no current guidelines to prevent Ewing sarcoma because the cause is unknown.
American Cancer Society
National Cancer Institute
Canadian Cancer Society
Ewings Cancer Foundation of Canada
Ewing family of tumors. American Cancer Society website. Available at:
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Accessed September 6, 2016.
Ewing sarcoma. EBSCO DynaMed website. Available at: Available at: http://www.dynamed.com/topics/dmp~AN~T114929/Ewing-sarcoma. Updated October 29, 2014. Accessed September 28, 2016.
Ewing sarcoma. St. Jude Children's Research Hospital website. Available at: https://www.stjude.org/disease/ewing-sarcoma.html. Accessed September 6, 2016.
Ewing’s sarcoma. Ortho Info—American Academy of Orthopaedic Surgeons website. Available at:
...(Click grey area to select URL)
Updated September 2011. Accessed September 6, 2016.
Ewing's sarcoma. UCSF Benioff Children's Hospital website. Available at: https://www.ucsfbenioffchildrens.org/conditions/ewings_sarcoma/index.html. Accessed September 6, 2016.
Miser ES, Goldsby RE, Chen Z, et al. Treatment of metastatic Ewing’s sarcoma/primitive neuroectodermal tumor of bone: evaluation of increasing the dose intensity of chemotherapy-a report from the children’s oncology group. Pediatr Blood Cancer. 2007;49(7):894-900.
Last reviewed May 2016 by Mohei Abouzied, MD
Last Updated: 5/28/2014
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