(Peripheral Primitive Neuroectodermal Tumors [PNET]; Ewing's Family of Tumors)
Pronounced: YOO-ingz sar-KOH-muh
by Diana Kohnle and Patricia Griffin Kellicker, BSN
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.
The causes of Ewing's 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. The following factors may increase your chance of developing Ewing's sarcoma:
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:
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 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 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 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.
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.
Ewing's sarcoma occurs for unknown reasons. There are currently no preventive measures to reduce the risk of developing the cancer.
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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/what.php . 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.ucsfhea... . Accessed June 24, 2013.
Solid tumors: Ewing sarcoma family tumors. Disease Information. St. Jude Children's Research Hospital website. Available at: http://www.stjude.... . Accessed June 24, 2013.
Last reviewed June 2013 by Igor Puzanov, MD; Michael Woods, MD
Last Updated: 5/11/2013