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Ewing's Sarcoma(Peripheral Primitive Neuroectodermal Tumors [PNET]; Ewing's Family of Tumors)

Pronounced: YOO-ingz sar-KOH-muh

Definition

Ewing's 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.

Leg and Pelvic Bones—Common Sarcoma Sites

Leg bones

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Causes

The causes of Ewing's sarcoma are not fully understood. Chromosomal rearrangement that alters the genes may be a factor.

Risk Factors

Because the causes of the cancer are unknown, risk factors are also not fully understood. The following factors may increase your chance of developing Ewing's sarcoma:

  • Race: Caucasians
  • Age—Teenagers
  • Gender—Male
Symptoms

Symptoms include:

  • Pain, redness, and swelling surrounding the tumor
  • Fever
  • Weight loss and reduced appetite
  • Fatigue
  • Paralysis and incontinence in cases where the tumor is near the spinal cord
  • Numbness, tingling, and paralysis caused by compression of nerves by the tumor
  • Difficulty breathing if the tumor is in the chest wall
Diagnosis

Your doctor will ask 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:

Bone Biopsy

Bone biopsy

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Treatment

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 follows. Chemotherapy then resumes for several months after surgery or radiation.

Chemotherapy

Chemotherapy drugs are used to kill tumor cells. The medications used to treat this tumor include vincristine (Oncovin) , dactinomycin (Actinomycin D), cyclophosphamide (Cytoxan), doxorubicin (Adriamycin), ifosfamide (Ifex), and etoposide (VePesid). Your doctor will prescribe a combination of these drugs to be given over a set time. This usually means every 2-4 weeks for several months.

Surgery

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

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.

Future Therapy

A combination of high-dose chemotherapy and stem cell transplant is being evaluated at certain cancer centers. This combination therapy is typically used in cases where the tumor is very resistant.

Prevention

Ewing's sarcoma occurs for unknown reasons. There are currently no preventive measures to reduce the risk of developing the cancer.

RESOURCES:

American Cancer Society
http://www.cancer.org

National Cancer Institute
http://www.cancer.gov

CANADIAN RESOURCES:

About Kids Health
http://www.aboutkidshealth.ca

Alberta Human Services
http://www.child.alberta.ca

References:

Ewing family of tumors. American Cancer Society website. Available at: http://www.cancer.org/docroot/CRI/CRI_2_3x.asp?rnav=cridg&dt=48. Accessed June 24, 2013.

Ewing’s sarcoma. American Academy of Orthopaedic Surgeons website. Available at: http://orthoinfo.aaos.org/topic.cfm?topic=A00082. Updated September 2011. Accessed June 24, 2013.

Ewing sarcoma. EBSCO DynaMed website. Available at: Available at: http://www.ebscohost.com/dynamed. Updated May 1, 2013. Accessed June 24, 2013.

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. Pediatric Blood Cancer. 2007;49:894-900.

Sarcomas: Ewing's sarcoma. The University of California, San Francisco Children's Hospital website. Available at: http://www.ucsfhealth.org/childrens/medical_services/cancer/sarcomas/conditions/ewings/signs.html. Accessed June 24, 2013.

Solid tumors: Ewing sarcoma family tumors. Disease Information. St. Jude Children's Research Hospital website. Available at: http://www.stjude.org/stjude/v/index.jsp?vgnextoid=174c061585f70110VgnVCM1000001e0215acRCRD&vgnextchannel=3b8fbfe82e118010VgnVCM1000000e2015acRCRD. Accessed June 24, 2013.



Last reviewed May 2014 by Mohei Abouzied, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.

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