Ewing Sarcoma

Ewing Sarcoma

Ewing sarcoma is a cancerous tumor that grows in bones or soft tissue near bones. It can develop anywhere in the body, but most commonly affects the arms, legs, ribs, spinal column, and pelvis.

Treatment for Ewing sarcoma involves a combination of chemotherapy, surgery, and/or radiation. With early diagnosis and proper treatment, many kids who develop it have a good chance of recovery.

Signs and Symptoms

Because most cases of Ewing sarcoma are in teens and kids older than 10, it often goes unnoticed or misdiagnosed as growing pains (due to its subtle, slow onset) or a sports injury because of similar symptoms.

Pain at the tumor site, as well as swelling or warmth, is common. The pain may be worse during exercise or at night, and a tender lump may form in the affected area a few weeks after pain begins. A tumor in a leg bone may cause a limp. A low-grade fever also can develop.

If cancer has spread, or metastasized, to other areas of the body like the lungs or bone marrow, symptoms may appear there as well. In some cases, the first sign of the disease is a broken arm or leg, which happens because the cancer has weakened the bone and made it vulnerable to a break.

If your child or teen has any of these symptoms, it's important to see a doctor as soon as possible.

Diagnosis

To diagnose Ewing sarcoma, a doctor will do a physical exam, take a detailed medical history, and order X-rays, an MRI (magnetic resolution imaging), or bone scans to find any changes in bone structure.

A tissue sample of the tumor (biopsy) must be done for a diagnosis to be made. If a needle biopsy is done, the doctor will use a long hollow needle to take a sample of the tumor. Local anesthesia (medicine that numbs the area so the person won't feel pain) usually is used. Or the doctor may order an open biopsy, in which a portion of the tumor is removed in the operating room by a surgeon while the child is asleep during the procedure under general anesthesia.

If a diagnosis of Ewing sarcoma is made, other imaging tests will be done to see if the cancer has spread to any other part of the body. Other exams will make sure that vital organs, such as the heart and kidneys, are working normally and able to tolerate treatment.

After treatment starts, repeating these tests will help determine how well treatment is working and whether the cancer is continuing to spread.

Chemotherapy/Radiation

Treatment for Ewing sarcoma depends on many things, including the size and location of the tumor and whether the disease has spread.

Most patients will undergo chemotherapy to kill cancer cells and shrink the cancer before surgical removal or radiation, and to prevent the spread of cancer cells to other parts of the body. Chemotherapy is also given after a tumor is removed to help ensure that cancer cells do not come back.

Chemotherapy usually lasts 6 months to a year. During treatment, children must stay in the hospital for a few days every 2 to 3 weeks. Some kids may need to be hospitalized between treatments for complications or side effects, like fever or infection. Chemo drugs are delivered intravenously (through a vein).

If surgery is needed, radiation may be given to shrink the tumor before surgical removal. Radiation also may be recommended in place of surgery if the tumor's location would make surgery too dangerous or difficult. During radiation, high-energy X-rays are directed at the tumor site to kill cancer cells and keep them from growing and multiplying.

Most kids undergoing radiation don't need to stay in the hospital overnight, but visit the hospital 5 days a week for several weeks instead.

Surgical Removal

After a course of chemotherapy, and possibly radiation, most tumors are removed surgically. Tissue that is removed may need to be replaced with a live tissue graft (from the patient's own body), donor tissue (usually from a bone bank), or artificial tissue (made from metal or other synthetic material).

If the cancer has spread to the nerves and blood vessels surrounding the original tumor, amputation (removing part of a limb along with the tumor) is often the only choice.

Side Effects

Amputation carries its own short-term and long-term side effects. It usually takes at least 3 to 6 months until a young person learns to use a prosthetic (artificial) leg or arm, and this is just the beginning of long-term psychological and social rehabilitation. But many children find they can resume their favorite activities in the long run.

With a tissue graft (donated or otherwise), a patient usually starts moving the affected body part almost immediately. For a child recovering from Ewing sarcoma of the leg, for example, physical therapy and rehabilitation for 6 to 12 months after surgery usually lets a child walk, initially with a walker or crutches, then without any assistive devices.

Early complications after surgery include infection and slow healing of the surgical wound. Also, a metal prosthetic device or the bank bone may need to be replaced as the body grows, although some prosthetics can be adjusted to fit as a child grows.

Other late problems might include fracture of the bank bone or failure of the bank bone to heal to the child's bone, which might require more surgery. Some kids will be limited in the types of sports they can play due to the risk of breakage.

Many of the medicines used in chemo also carry the risk of both short-term and long-term problems. Short-term effects include anemia, abnormal bleeding, and increased risk of infection due to destruction of the bone marrow, as well as kidney, liver, or heart damage.

Some drugs carry a risk of bladder inflammation and bleeding into the urine. Years after chemotherapy for Ewing sarcoma, patients have an increased risk of developing other cancers and may have problems with fertility.

Prognosis

If Ewing sarcoma is diagnosed early and has not spread, a child has up to a 70% chance of being cured of the cancer.

When the disease has spread, successful treatment is more difficult. However, with the potent combination of treatments currently available — and powerful new therapies on the horizon — the outlook for kids with advanced disease is improving.

Reviewed by: Christopher N. Frantz, MD
Date reviewed: September 2014

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Note: All information is for educational purposes only. For specific medical advice, diagnoses, and treatment, consult your doctor.
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