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:

        
  • X-rays
  • Bone scans
  • CT scan
  • MRI scan
  • PET/CT scan —to evaluate the metabolic activity of tissue
  • Your bodily fluids and tissues may need to be tested. This can be done with:

        
  • Blood tests
  • Biopsy
  • 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.