What is Ewing's sarcoma?

Causes and Symptoms

The specific cause of Ewing’s sarcoma

is unknown, but it may be associated with recurrent trauma, metal implants, congenital anomalies, unrelated tumors, or exposure to ionizing radiation. Approximately 90 percent of patients are between five and twenty-five years of age; rarely are patients younger than five or older than forty.

The initial symptom is pain, discontinuous at first and then intense, in the long bones, vertebra, or pelvis. Swelling may follow. Neurological signs involving the nerve roots or spinal cord
depression are characteristic of nearly one-half of patients with involvement of the axial skeleton. Weight loss may occur, with remittent fever and mild anemia.

The phases of Ewing’s sarcoma are based on degree of metastasis: the local phase (a nonmetastatic tumor), the regional phase (lymph node involvement), and the distant phase (involvement of the lungs, bones, and sometimes the central nervous system).

Treatment and Therapy

Patients are of two types, those with localized tumors and those with metastasized tumors. Depending on where the tumor is located, the treatment of Ewing’s sarcoma is complex in all stages of disease and requires a multidisciplinary perspective. It is best treated when diagnosed early. Obtaining a bone biopsy is recommended in nearly all cases.

Surgery may be used to remove a tumor, followed by chemotherapy administered to kill any remaining cancer
cells. Radiation may be prescribed to kill cancer cells and shrink tumors.

Perspective and Prospects

Ewing’s sarcoma is one of the most malignant of all tumors. It may be localized or metastasize to the lungs and other bones. The primary tumor can be controlled by irradiation, but the prognosis is poor. Often, amputation is not justifiable. Recent developments in multiagent chemotherapy, however, are encouraging. Long-term survival of patients with Ewing’s sarcoma is 50 to 70 percent or more with localized disease; the rate drops to less than 30 percent for metastatic disease.

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