Ewing Sarcoma

Also called: Askin tumor, Ewing’s sarcoma, peripheral primitive neuroectodermal tumor, pPNET

What is Ewing sarcoma?

Ewing sarcoma is a cancerous tumor that grows in the bones or in the tissue around bones (soft tissue)—often the legs, pelvis, ribs, arms or spine. Ewing sarcoma can spread to the lungs, bones and bone marrow.

How common is Ewing sarcoma?

Ewing sarcoma is the second most common type of bone cancer in children, but it is very rare. About 200 children and young adults are found to have Ewing sarcoma each year in the United States.

  • About half of all Ewing sarcoma tumors occur in children and young adults between ages 10 and 20.
  • This type of cancer is rarely found in African-Americans and Asian-Americans.
  • It affects slightly more boys than girls.
  • It does not appear to be inherited (passed down in families).
  • Although not often seen, Ewing sarcoma can occur as a second cancer, especially in patients treated with radiation therapy.

What are the symptoms of Ewing sarcoma?

If your child has Ewing sarcoma, the following symptoms may be present:

  • Swelling and soreness around the tumor area (commonly mistaken for a sports injury or the “bumps and bruises” every child gets)
  • A low fever that at first may seem to be caused by an infection
  • Bone pain, especially pain that worsens during exercise or at night
  • Limping, which is caused by a tumor on a leg bone

How is Ewing sarcoma treated?

  • Chemotherapy (“chemo”)—is usually the first step in treating Ewing sarcoma. It uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells:
    • Chemo may be injected into the bloodstream, so that it can travel throughout the body.
    • Combination therapy uses more than one type of chemo at a time.
  • Surgery—may be done after several weeks or months of chemo have reduced the cancer to the point where surgery can be most effective.
    • Surgeons remove as much of the tumor as possible.
    • Sometimes the surgeons will graft (add on) bone or tissue (from either the patient or a donor) to replace diseased bone and tissue that have been removed. An artificial bone, called an implant, may also be used.
    • For some patients, amputation (surgical removal of an arm or leg) is required to ensure that the tumor is completely removed.
  • Radiation therapy—is used to kill or decrease cancer cells that cannot be removed surgically. This is followed by more chemo to kill any remaining cells. Radiation therapy uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing:
    • External radiationuses machines outside the body to deliver the X-ray dose.
    • Internal radiationuses needles, seeds, wires or catheters (tubes) to deliver the radiation directly into or close to the cancer.

What are the survival rates for Ewing sarcoma?

  • About 70 percent of children with Ewing sarcoma are cured.
  • Teens aged 15 to 19 have a lower survival rate of about 56 percent.
  • For children diagnosed after their disease has spread, the survival rate is less than 30 percent.
  • Children whose tumors are larger or located in the pelvis, ribs or spine are less likely to be cured.

Why choose St. Jude for your child’s Ewing sarcoma treatment?

  • St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.
  • St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
  • The nurse-to-patient ratio at St. Jude is unmatched—averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
  • St. Jude has a new clinical trial for patients with Ewing sarcoma that investigates new drug combinations at the beginning and end of therapy to improve survival.
  • New radiation treatments such as intensity-modulated radiation therapy (IMRT) and proton beam radiation therapy may be used to decrease the side effects of treatment.
  • St. Jude has a specialized team of experts who perform limb-salvage procedures (surgery) in children with bone tumors such as Ewing sarcoma.
  • New imaging techniques allow St. Jude to better define the effects of treatment in children with Ewing sarcoma. These techniques include contrast-enhanced ultrasound and diffuse weighted imaging MRI.
  • Working with European researchers, St. Jude scientists have sequenced and are analyzing the genetic features of more than 100 children with Ewing sarcoma to find possible new targets for treating the disease.

Associated Clinical Trials

ESFT13: Therapeutic Trial for Patients with Ewing Sarcoma Family of Tumors and Desmoplastic Small Round Cell Tumors

Combination Chemotherapy with or without Sorafenib Tosylate and Bevacizumab in Treating Patients with Ewing Sarcoma or Desmoplastic Small Round Cell Tumors

Diseases Treated:

Ewing Sarcoma Family of Tumors and Desmoplastic Small Round Cell Tumors

Eligibility:

  • Diagnosis of Ewing sarcoma and/or desmoplastic small round cell tumor (DSRCT)
  • Less than 25 years of age
  • Has not received previous treatment with radiation therapy or chemotherapy. Emergent radiation therapy to preserve vital organ function is permitted.
View Trial

SBRT1: Stereotactic Radiation Therapy for Pediatric Sarcomas

A Phase II Study of Hypofractionated Stereotactic Radiotherapy in the Treatment of Metastatic Pediatric Sarcomas of Bony Sites

Diseases Treated:

Metastatic sarcomas

Eligibility:

  • Participant has histologically or cytologically confirmed diagnosis:
    • Nonrhabdomyosarcoma of soft tissue
    • Ewing sarcoma
    • Osteosarcoma at any site
  • Participant must be greater than 3 years of age and < 40 years of age.
  • Participant has not had any prior radiotherapy to the treatment site.
View Trial

SJGD2: Monoclonal Antibody Therapy in Treating Younger Patients with Relapsed or Refractory Neuroblastoma, Osteosarcoma, or Melanoma

A Phase I Trial of the Humanized Anti-GD2 Antibody(HU14.18K322A) in Children and Adolescents with Neuroblastoma, Osteosarcoma, Ewing Sarcoma and Melanoma

Diseases Treated:

Neuroblastoma, osteosarcoma, melanoma or Ewing sarcoma

Eligibility:

  • Up to 21 years of age
  • Diagnosis of neuroblastoma, melanoma, osteosarcoma  or Ewing sarcoma that has come back or did not respond to previous treatment
  • Any uncontrolled infection
View Trial

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