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The University of Chicago Medicine - Comer Children's Hospital

Ewing Sarcoma: Frequently Asked Questions

Here are answers to some questions frequently asked by parents of children with Ewing sarcoma (Ewing’s sarcoma):

Exactly what triggers Ewing sarcoma is not known. But we do know that the cancer cells in Ewing sarcoma almost always have a specific chromosomal abnormality causing normal cells to grow out of control. It is important to know that this chromosomal abnormality is not something a child inherits; it seems to be an "accident" that occurs in a cell sometime after birth. Often, the sarcoma cells are tested for this particular chromosomal abnormality, providing a tool that confirms the diagnosis of Ewing sarcoma.

There are no known risk factors for Ewing sarcoma and no known way to prevent the disease.

It’s rare for other family members to be affected. We always analyze family history for each new patient. Occasionally a genetic problem is suspected. In that case, the patient and certain family members are sometimes referred to our Pediatric Familial Cancer Center to be tested for inherited defects that might predispose them to cancer.

Once the diagnosis has been confirmed, your oncologist will assign a stage to the tumor. Ewing sarcomas are staged as either localized or metastatic (spread to other parts of the body).

If the tumor cells have metastasized (spread), they can be found in other areas of the body including the bone marrow, lungs, kidneys, heart, adrenal gland and other soft tissues.

Ewing sarcoma treatment often includes surgery for the initial biopsy, and to remove the primary tumor that is visible. When the tumor occurs in a bone, this is usually done with bone grafts, limb-sparing procedures and reconstruction. In cases where the primary tumor cannot be completely removed, radiation therapy is often used with or in place of surgery. Because radiation treatment is very effective, amputation is rarely necessary for Ewing sarcoma.

Chemotherapy, before and after surgery, is a critical part of the comprehensive and multidimensional treatment of Ewing sarcoma. It is sometimes given before surgery in order to shrink the tumor, which can make surgical removal easier to accomplish. Chemotherapy is also given after surgery, even if there is no other evidence of tumor left behind. This is done because we know that microscope amounts of Ewing sarcoma remain after surgery. Chemotherapy is used to eradicate these cells to minimize the chance that the cancer will come back at other sites. Chemotherapy for Ewing sarcoma is nearly always given intravenously (IV) through a catheter -- called a "central line." Chemotherapy is usually given over a period of 2 to 5 days in the hospital. Each of these 2 to 5 day cycles of chemotherapy is given every 2 to 3 weeks. Usually, about 14 cycles of chemotherapy are used.

Radiation is often given to sites where the Ewing sarcoma is visible. In situations where surgery cannot be used, radiation treatments can effectively control the tumor and prevent the cancer from growing. In some cases, radiation therapy is used after a primary tumor is removed in an effort to get rid of any cancer cells that might have been on the margin or edge of the tumor removal site.

Chemotherapy for Ewing sarcoma is usually given in cycles, with each cycle covering a period of 2 to 5 days in the hospital. A new cycle is given every 2 to 3 weeks. Usually, about 14 cycles of chemotherapy are needed. The total duration can be from 30 to 45 weeks. At some stage during the treatment, surgery is often performed to remove the visible tumor. The recovery period in the hospital varies from patient to patient, lasting from several days to 1 to 2 weeks. Radiation therapy, if needed, is given 5 days per week for 4 to 6 weeks. It is administered in the outpatient treatment center. Chemotherapy is often modified or delayed during surgery or radiation therapy.

Chemotherapy drugs not only attack the cancer cells, they can injure normal tissue and blood-forming cells. For this reason, your child may experience some of the common side effects of chemotherapy including low blood counts (causing anemia), nausea, vomiting, hair loss, mouth sores, irregular periods (girls) and inability to produce sperm (boys). Radiation therapy may put children at a risk of developing a second malignant tumor later in life. Your treatment team will do everything they can to reduce or prevent side effects of treatment.

Patients and their parents should talk to their doctor about the reproductive risks associated with cancer treatment. In certain situations, there are options available to try to preserve fertility, such as with sperm banking. These programs are available at the University of Chicago Medicine Comer Children’s Hospital.

Scientists and researchers are continually making progress in the fight against cancer. At any given time, many clinical trials of new drugs and protocols for childhood cancers are being tested. Across North America, most children with cancer are treated in a clinical research trial. Your physician only recommends a clinical trial when your care team believes it offers the best chance for a cure. Even if your treating physician recommends treatment on a clinical trial, you always have the option to choose the "standard of care" treatment that does not involve research.

The prognosis for survival varies depending on the stage of the tumor when it is detected and treated and other factors. The survival rate for Ewing sarcoma patients who undergo chemotherapy and surgery, and who have no metastases, is 60 to 70 percent. About 20 to 25 percent of children with metastatic Ewing sarcoma are cured of the disease.

Unfortunately, Ewing sarcoma can come back after treatment. If Ewing sarcoma comes back, it can often be treated with surgery, additional radiation therapy, and additional chemotherapy, including more experimental medicines and (possibly) stem cell transplant.

Behind the scenes, University of Chicago physicians and scientists are studying the biological and genetic basis of sarcomas and other cancers. Their findings may contribute to more effective ways to diagnose and treat Ewing sarcoma and to prevent the recurrence of this and other cancers that affect children and adults. Read more about sarcoma research under way at the University of Chicago.