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Ewing sarcoma

Definition

Ewing sarcoma is a malignant (cancerous) bone tumor that affects children.

Alternative Names

Ewing family of tumors; Primitive neuroectodermal tumors (PNET)

Causes

Ewing sarcoma can occur anytime during childhood and young adulthood. But it usually develops during puberty, when bones are growing rapidly. It is 10 times as common in Caucasian children as in African American, African, and Asian children.

The tumor may start anywhere in the body. Most often, it starts in the long bones of the arms and legs, the pelvis, or the chest. It can also develop in the skull or the flat bones of the trunk.

The tumor often spreads (metastasizes) to the lungs and other bones. At the time of diagnosis, spread is seen in about one-third of children with Ewing sarcoma.

In rare cases, Ewing sarcoma occurs in adults.

Symptoms

There are few symptoms. The most common is pain and occasionally swelling at the site of the tumor.

Children may also break a bone at the site of the tumor after a minor injury.

Fever may also be present.

Exams and Tests

If a tumor is suspected, tests to locate the primary tumor and any spread (metastasis) often include:

Treatment

Treatment often includes a combination of:

Support Groups

The stress of illness can be eased by joining a cancer support group. Sharing with others who have common experiences and problems can help you not feel alone.

Outlook (Prognosis)

How well a patient does depends on:

  • The location of the tumor
  • If the cancer has spread

The best chance for cure is with a combination of treatments that includes chemotherapy plus radiation or surgery.

Possible Complications

The treatments needed to fight this disease have many complications. Discuss these with your doctor.

When to Contact a Medical Professional

Call your health care provider if your child has any of the symptoms of Ewing sarcoma. An early diagnosis can increase the possibility of a favorable outcome.

References

Anderson ME, Randall RL, Springfield DS, Gebhardt MC. Sarcomas of bone. In: Niederhuber JE, Armitage JO, Doroshow JH, et al., eds. Abeloff's Clinical Oncology. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2013:chap 92.

Cote GM, Choy E. Update in treatment and targets in Ewing sarcoma. Hematol Oncol Clin N Am. 2013;27:1007-1019.

National Cancer Institute: PDQ Ewing Sarcoma Treatment. Bethesda, Md: National Cancer Institute. Date last modified: October 18, 2013. Available at: http://cancer.gov/cancertopics/pdq/treatment/ewings/HealthProfessional. Accessed: March 23, 2014.

National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines): Bone cancer. Version 1.2014. Available at: http://www.nccn.org/professionals/physician_gls/pdf/bone.pdf. Accessed March 23, 2014.

Review Date:3/23/2014
Reviewed By:Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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