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 10 times as common in Caucasian children as in African-American, African, and Asian 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.

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

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.

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
• MRI of the tumor
• X-ray of the tumor

Treatment

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

• Chemotherapy
  • Cisplatin
  • Doxorubicin
  • Etoposide
  • Ifosfamide
  • Methotrexate
• 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.

Prevention

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References

Baker LH. Bone tumors: primary and metastatic bone lesions. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 212.

National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Bone Cancer. National Comprehensive Cancer Network; 2010. Version 1.2010.