Localized Ewing tumors

After the Ewing tumor is diagnosed, the first treatment is chemotherapy. It is called neoadjuvant chemotherapy because it is given before any surgery or radiation therapy. As already described, treatment is usually a combination of vincristine, doxorubicin, and cyclophosphamide, alternated with ifosfamide and etoposide, although other combinations of the same drugs are also effective.

After at least 8 to 12 weeks of chemotherapy, imaging tests such as CT, MRI, PET, or bone scans are done to see if the tumor can be surgically removed. If so, surgery is done at this point. If surgery is not an option or if after the operation cancer cells are found at the edges of the surgery specimen (meaning cancer cells may have been left behind), radiation therapy is used. In either case, this is typically followed by more chemotherapy, which is given for several more months. Chemotherapy is not interrupted during radiation therapy.

Metastatic Ewing tumors

Patients who have metastatic disease when they are first diagnosed are harder to treat than patients with localized disease. The prognosis is better when the metastases are limited to the lungs, as opposed to when the cancer has spread to other bones or to the bone marrow.

Chemotherapy is the first treatment, often using a more intense regimen than would be used if the cancer was localized. After a few months, tests such as CT or MRI scans, bone or PET scans, and/or bone marrow biopsies are done to see how the cancer has responded to treatment.

If the cancer has only spread to a few small areas, the primary tumor and all known areas of metastases may be removed at this point. Other options, such as surgery plus radiation therapy or radiation therapy alone to all known metastatic sites, including the lungs, might also be tried.

After treatment to control the primary tumor, chemotherapy is given again for several months.

To improve the outcome for these patients, an approach consisting of very intensive chemotherapy followed by a peripheral blood stem cell transplant is being studied at several cancer centers. For more information on this procedure, see the "High-dose chemotherapy and stem cell transplant" section of this document.

Because these tumors can be hard to treat, clinical trials of newer treatments may be a good option in many cases.

Ewing tumors that recur (come back) after treatment

Recurrence of Ewing tumors after treatment is less likely than in the past, but it can happen. If the tumor does recur, treatment may depend on a number of factors, including

• the size and location of the tumor
• whether it has spread to different parts of the body
• what types of treatment the child had before
• how long it has been since treatment

Chemotherapy, surgery, and radiation therapy may be used to treat recurrent tumors. Doctors are also studying the use of high-dose chemotherapy followed by a peripheral blood stem cell transplant, although it is not yet clear how useful this is. Because treatment of these tumors can be difficult, clinical trials of newer treatments may be a good option.

Last Medical Review: 08/20/2009
Last Revised: 08/20/2009