Treatment

Differential diagnosis

Other entities that may have a similar clinical presentation include osteomyelitis, osteosarcoma and eosinophilic granuloma. Soft tissue neoplasms such as malignant fibrous histiocytoma that erode into adjacent bone may also have a similar appearance.

Most patients with Ewing tumors are treated in clinical trials according to national treatment guidelines called protocols.

The types of treatment that can be used in TEF include:

§ Chemotherapy

§ Surgery

§ Radiation therapy

§ High-dose chemotherapy followed by a stem cell transplant

Chemotherapy is almost always the first treatment. Localized therapy (surgery and/or radiation therapy) is next, often followed by more chemotherapy. A stem cell transplant might be an option for some patients with Ewing tumors that are unlikely to be cured with other treatments.

Surgery for Ewing sarcoma involves surgical removal of the tumor, bone/skin grafts, limb salvage procedures, amputation, and/or reconstruction. The type of surgery will depend on the size and location of the tumor, and whether the cancer has spread.

Treatment lengths vary depending on location and stage of the disease at diagnosis. Radical chemotherapy may be as short as 6 treatments at 3 week cycles; however most patients will undergo chemotherapy for 6–12 months and radiation therapy for 5–8 weeks.

Note!Transplantation of normal stem cells from another person is used to help restore normal blood production in patients whose own ability to make any or all of these blood cells has been compromised by cancer, intensive cancer treatment, or other types of damage or abnormality. The use of cells from another individual is called allogeneictransplantation. Stem cells collected form patients themselves prior to intensive treatment can also be used to supplement the recovery of the patient's own cells after particularly aggressive course of chemotherapy or radiation therapy. The use of a patient's own cells is referred to as autologous transplantation.

In the past, this type of treatment was often called a bone marrow transplant.

If a stem cell transplant is considered as part of the initial treatment plan for a Ewing tumor, the patient first gets standard doses of chemotherapy, then local treatment of the tumor (surgery and/or radiation therapy), followed by high-dose chemotherapy and a stem cell transplant.

In the past, the stem cells were often collected from the child’s bone marrow, which required a minor operation. But researchers have found that these cells can be taken from the bloodstream using a procedure similar to a blood donation. Instead of going into a collecting bag, the blood goes into a special machine that filters out the stem cells and returns the other parts of the blood to the person’s body. The stem cells are then frozen until the transplant. This may need to be done more than once. Once the stem cells have been frozen and stored, the child gets high-dose chemotherapy, sometimes along with radiation therapy. When the treatment is finished, the patient’s stem cells are thawed and returned to the body in a blood transfusion. The stem cells travel through the bloodstream and settle in the bone marrow. Over the next 3 or 4 weeks, they start to make new, healthy blood cells.

Until this happens, the child is at high risk of infection because of a low white blood cell count, as well as bleeding because of a low blood platelet count. To avoid infection, protective measures are taken, such as using special air filters in the hospital room and having visitors wear protective clothing. Blood and platelet transfusions and treatment with antibiotics may also be used to prevent or treat infections or bleeding problems.