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Ewing's Sarcoma (ES)
Sunday, 31 August 2003
Tuesday, 28 October 2003

What

Ewing’s sarcoma (ES) is the second most common type of primary bone cancer in children ten years old and younger. Most commonly, the initial site of cancer is the bone. However, less than 10% of the time the Ewing’s sarcoma develops in the soft tissue surrounding the bone. A Ewing’s sarcoma tumor is composed of abnormal round blue cells. The cancer can spread from the bone to other organs and tissues through the blood. Ewing’s sarcoma typically affects the growth plate areas of long bones such as the arms and legs. It also commonly occurs in flat bones of the shoulder and pelvis. Ewing’s sarcoma is rarely found in the spine and the small bones of the feet and hands.

Who

Ewing’s sarcoma is a very rare disease. The incidence in the United States is approximately 2.1 cases per 1 million children each year. The most common age of diagnosis is in the 2nd decade of life, usually between the ages of 10 and 20 years. Ewing’s sarcoma is more common in males, and the ratio is 3 males to 2 females. All races are affected by this disease; however, it is very rare in African-Americans.

Signs and Symptoms

Children with Ewing’s sarcoma may have the following symptoms (and so sometimes it can be difficult to distinguish from infection): 1. pain (mild and intermittent initially) 2. fever 3. swelling 4. bone fracture at tumor site 5. a bump that can be felt, sometimes warm to touch

Possible Causes

The cause of Ewing’s sarcoma is currently not known, although often a specific chromosomal rearrangement is found in the tumor cells (EWS-FLI 1 being the most common translocation. This translocation is present in 90-95% of ES tumors.)

Diagnosis

The extent of the primary tumor is best visualized with an MRI. A plain radiograph of the primary site is also obtained at time of initial presentation and often shows an “onion peel” appearance of the affected bone which indicates destruction of the bone. Since the most common sites of metastases are the lung, bone and bone marrow, a bone scan, chest CT scan, and bone marrow biopsies are done at time of initial presentation to evaluate areas of potential spread of the cancer. For definitive diagnosis, a biopsy of the tumor must be performed and sent to the laboratory for microscopic examination and for chromosomal testing of the tumor. The initial surgical biopsy of the tumor is extremely important because if not done by a skilled surgeon, it can result in a pathologic fracture or contamination of surrounding tissue with tumor cells. A fine needle aspirate of the tumor is not an acceptable diagnostic technique because of the special testing that must be done on the tissue to make the correct diagnosis since it can look similar to other tumors (specifically other small, blue round cell tumors) on first inspection under the microscope.

Treatment

Treatment of children with Ewing’s sarcoma involves treatment of the initial tumor as well as treatment of the cancer that has spread to other locations. Chemotherapy, surgical resection and radiation therapy can be used to treat the cancer. The goal of surgery is to completely excise the tumor and maintain the maximum level of functional use of the limb. Radiation therapy may be used if the tumor is unresectable or complete surgical resection would result in mutilation of the limb. Chemotherapy is used to treat microscopic and gross metastatic disease, as well as, decreasing the local tumor size. Prior to the use of chemotherapy, the survival of patients with ES was approximately 10%.

Prognosis

The prognosis of a child with Ewing’s sarcoma is variable and depends on many factors. Children with small localized tumors have up to 75% cure rate. Primary tumor site is also important for prognosis for those with localized disease: more favorable prognosis is associated with tumor in distal bones, ribs, soft tissue sites and least favorable being the pelvis. It should also be noted, however, that the favorable locations are also associated with a better chance of complete surgical resection. Other poor prognostic features include those with the spread of the cancer to distant sites have a lower survival rate. An elevated level of the blood test lactose dehydrogenase level (LDH) drawn at the time of the initial diagnosis has been associated with a poorer prognosis. Recurrence of the disease is another sign of lower rate of survival and the sooner the recurrence, the more serious the concern. Lastly, response of the tumor to chemotherapy by evaluation under the microscope is also another important prognostic variable.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

American Cancer Society. Search for Ewing sarcoma.
A great link with information about the disease with a nice glossary of medical terms. Also with information about support groups for cancer patients.

Children’s Cancer Web
for children and families affected by the disease.

Bone Tumor. Org
A nice summary of the disease process with links to other resources.

The Cure Our Children Foundation
An excellent website with information about the disease as well as support for families affected by the disease.

Teens Living with Cancer
A nice website geared toward providing information and support for teens with cancer.

Google Search for Ewing's Sarcoma (ES)

References and Sources

www.cancer.org/docroot/home/index.asp www.cancerindex.org/ccw/guide2e.htm www.bonetumor.org/tumors/pages/page141.html