Ewing's sarcoma

Definition

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

Alternative Names

Ewing's family of tumors; Primitive neuroectodermal tumors (PNET)

Causes, incidence, and risk factors

Ewing's sarcoma can occur any time during childhood and young adulthood, but usually develops during puberty, when bones are growing rapidly. It is uncommon in African-American, African, and Chinese children.

The tumor may arise anywhere in the body, usually in the long bones of the arms and legs, the pelvis, or the chest. It may also develop in the skull or the flat bones of the trunk.

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 seemingly minor injury (this is called a "pathologic fracture"). Fever may also be present.

The tumor often spreads (metastasis) to the lungs and other bones. Metastasis at the time of diagnosis is present in approximately one-third of children with Ewing's sarcoma. Rarely, Ewing's sarcoma can occur in adults.

Symptoms

• Fever (can occur)
• Pain at the site of the tumor
• Swelling at the site of the tumor (occasionally)

Signs and tests

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

• Biopsy of the tumor
• Bone scan
• Chest x-ray
• CT scan of the chest
• Skeletal x-ray

Treatment

Treatment should be done by a cancer specialist (oncologist) and often includes a combination of:

• Chemotherapy
  • Cyclophosphamide
  • Vincristine
  • Dactinomycin
  • Doxorubicin
  • Ifosfamide
  • Etoposide
• Radiation therapy to the tumor site
• Surgical excision (removal) of the primary tumor

Support Groups

For additional information and resources, see cancer support group.

Expectations (prognosis)

The prognosis depends on the location of the tumor, and whether or not the cancer has spread. The best chance for cure is with a combination of treatments that includes chemotherapy plus radiation or surgery, provided at an institution that frequently treats this type of cancer.

Complications

The treatments needed to fight this disease have many complications, which should be discussed on an individual basis.

Calling your health care provider

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

References

Lahl M, Fisher VL, Laschinger K. Ewing's sarcoma family of tumors: an overview from diagnosis to survivorship. Clin J Oncol Nurs. 2008:12(1):89-97.

Skubitz KM, D'Adamo DR. Sarcoma. Mayo Clin Proc. 2007:82(11):1409-1432.