Treatment

In general, cancer in children is uncommon, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That’s why more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare standard treatments (the best treatments available) with newer treatments that may be more effective. Investigating new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track progress.

To take advantage of these newer treatments, all children with cancer should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children with cancer and have access to the latest research. A doctor who specializes in treating children with cancer is called a pediatric oncologist. Many times, a team of doctors treats a child with cancer. Pediatric cancer centers often have extra support services for children and their families, such as nutritionists, social workers, and counselors. Special activities for kids with cancer may also be available.

Children and teenagers with EFT should be treated in clinical trials specifically designed for their disease. A typical treatment plan for EFT consists of systemic therapy (therapies that treat the entire body, such as chemotherapy or stem cell transplantation [SCT]) combined with localized therapy. Localized therapy is therapy to treat the tumor itself, such as surgery and radiation therapy. When more than one treatment is used, it is called combination therapy. Doctors make treatment decisions based on the stage of the disease and the age of the child, while trying to avoid or reduce long-term side effects of treatment.

Descriptions of the most common treatment options for EFT are listed below.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. Some people may receive chemotherapy in their doctor’s office; others may go to the hospital. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a specific time. The doctor may use surgery or radiation therapy (see below) first and then give chemotherapy to eliminate any remaining EFT cells.

Chemotherapy for EFT is usually injected into a vein or muscle; it is rarely given by mouth. Patients with EFT may receive vincristine (Oncovin), cyclophosphamide (Cytoxan, Neosar), and doxorubicin (Adriamycin, Rubex), ifosfamide (Ifex), and etoposide (VePesid, Etopophos). For patients with EFT that has not spread to other parts of the body, the standard schedule is to receive chemotherapy every two weeks. People with metastatic disease may also be treated with the above medications and dactinomycin (Cosmegen).

The side effects of chemotherapy depend on the individual and the dose used, but can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.

Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your child's doctor is often the best way to learn about the medications prescribed for your child, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

Surgery

When it is possible, surgical removal of the tumor should be performed after chemotherapy (called neoadjuvant chemotherapy). A surgical oncologist is a doctor who specializes in treating cancer using surgery. The doctor may perform surgery to remove the cancer and some surrounding tissue. Surgery may also be needed to remove any remaining cancer cells after chemotherapy or radiation therapy. Learn more about cancer surgery.

Often a tumor can be removed without causing disability. If the cancer occurs in the arm or leg, surgery to remove much of the bone may affect the limb's ability to function.

Bone grafts from other parts of the body may help to reconstruct a limb. A prosthesis (artificial limb) made of metal or plastic bones or joints can replace lost tissue. Physical therapy after surgery can help the child learn to use the limb again. Learn more about rehabilitation.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other high-energy particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. For EFT, radiation therapy is used when surgery is not possible or did not remove all cancer cells, as well as in cases when chemotherapy was not effective. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation therapy is given using implants, it is called internal radiation therapy or brachytherapy. Intraoperative radiation therapy (radiation given inside the body during surgery) is being studied in clinical trials.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. In the long term, radiation therapy can also interfere with normal bone growth and is associated with the development of a secondary cancer. Learn more about radiation therapy.

Stem cell transplantation/bone marrow transplantation

This is an experimental approach that is still under evaluation in the treatment of EFT.

A stem cell transplant is a medical procedure in which diseased bone marrow is replaced by highly specialized cells, called hematopoietic stem cells. Hematopoietic stem cells are found both in the bloodstream and in the bone marrow. Today, this procedure is more commonly called a stem cell transplant, rather than bone marrow transplant, because blood stems cells are typically what is being transplanted, not the actual bone marrow tissue.

There are two types of stem cell transplantation depending on the source of the replacement blood stem cell: allogeneic (ALLO) and autologous (AUTO). AUTO transplantations are used to treat EFT.

In an AUTO transplant, the patient’s own stem cells are used. The stem cells are obtained from the patient when he or she is in remission from previous treatment. The stem cells are frozen until they are needed, usually after the high-dose treatment (explained below) is completed.

The goal of transplantation is to destroy cancer cells in the marrow, blood, and other parts of the body and have replacement blood stem cells create healthy bone marrow. In most stem cell transplants, the patient is treated with high doses of chemotherapy and/or radiation therapy to destroy as many cancer cells as possible. This also destroys the patient’s bone marrow tissue and suppresses the patient’s immune system. After the high-dose treatment is given, blood stem cells are infused into the patient’s vein to replace the bone marrow and restore normal blood counts.

Before recommending transplantation, doctors will talk with the patient about the risks of this treatment and consider several other factors, such as the type of cancer, results of any previous treatment, and patient’s age and general health.

In SCT, replacement cells engraft (begin to make new blood cells) and turn into healthy, blood-producing tissue in 10 days to three weeks. Destroying the patient’s own marrow reduces the body’s natural defenses, temporarily leaving the patient at an increased risk of infection. Until the patient’s immune system is back to normal, patients may need antibiotics and blood transfusions.

In an AUTO transplant, there is little risk of tissue rejection because the replacement stem cells are the patient’s own cells. However, there is a risk in an autologous transplant that some of the cells that are put back into the patient could still be cancerous. Learn more about bone marrow and stem cell transplantation.

Recurrent EFT

If the tumor comes back after initial treatment, the next round of treatment depends on where and when the cancer recurred and how it was first treated. The doctor may use chemotherapy, including cyclophosphamide and topotecan (Hycamtin), radiation therapy, and/or may surgically remove new tumors. SCT may also be recommended.

New drugs that are being tested in clinical trials for effectiveness against EFT may also be used.

Find out more about common terms used during cancer treatment.