About this Cancer
Ewing’s sarcoma is a cancerous bone tumor affecting children and young adults. It gets its name from Dr. James Ewing, the doctor who first described the tumor in the 1920s. Ewing’s sarcoma may arise anywhere in the body, but it usually originates in the long bones of the arms and legs, the pelvis, or the chest. Though more rare, it may also develop in the skull or flat bones of the trunk. While it is most often a bone tumor, Ewing’s sarcoma can develop in the soft tissue near bones as well. Primitive neuroectodermal tumors (PNET) are molecularly related tumors that often arise outside of the bone. Treatment is the same as Ewing’s sarcoma.
While Ewing’s sarcoma can occur anytime during childhood, it develops most commonly during puberty, when bones are growing rapidly. This type of cancer is uncommon in African-American, African, and Chinese children.
Symptoms of Ewing’s sarcoma include: pain in the bone where the tumor is located; swelling in the area of the tumor, and tenderness to touch; occasionally, a fever may develop; or the child may have a broken bone after a seemingly minor fall or accident.
The doctor may order a variety of tests to diagnose Ewing’s sarcoma. An X-ray of the painful part of the bone may be done to see if a tumor is present. Other tests administered to obtain an image of the tumor and determine if it has spread beyond the bone include: a CT (computerized tomography) scan, an MRI (magentic resonance imaging) scan, a chest X-ray, and a bone scan (scintigraphy). A bone scan detects areas of increased or decreased bone metabolism and can identify abnormal processes involving the bone, including a tumor.
These imaging tests are typically noninvasive and painless. A biopsy will be needed to make the diagnosis. A biopsy is a surgical procedure used to obtain a sample of the tumor so that it can be examined under a microscope and tested in other ways.
Treatment is usually a combination of therapies including surgery, chemotherapy, and radiation therapy. The exact type of treatment a patient receives depends upon the size, position, and spread of the tumor.
Chemotherapy, treatment with anticancer drugs, is usually done first to shrink the primary or main tumor. A specialist, called an orthopedic surgeon, will then remove the tumor surgically.
The surgeon will make every effort to remove the tumor without causing too much damage to the limb. This is called limb-sparing surgery. In this type of surgery, the diseased part of the bone is removed and replaced with an artificial piece (prosthesis) or piece of bone from a bone bank (bone graft).
After this type of surgery, the patient usually regains nearly normal use of the limb. If the child is growing, and an artificial piece was used to replace the diseased bone, additional surgeries may be required to lengthen the artificial bone as the child grows. The doctor may advise against contact sports as well, because, if the bone graft or prosthesis is damaged, another major operation may be needed to repair or replace it.
If the tumor is in one of the main bones of the arm or leg and has affected surrounding blood vessels and nerves, surgery to remove the entire limb (amputation) may be necessary. Though the prospect of amputation is alarming to families and patients, children fitted with artificial limbs are usually able to resume normal activities and even play sports. Artificial limbs are precisely fitted to each child and are adjusted regularly as the child grows.
Radiation therapy, targeted X-rays that kill cancer cells, may be given if surgery to remove the tumor is not possible. In addition , chemotherapy will be given following surgery or radiation therapy to destroy any tumor cells that may have spread to other parts of the body.
Most patients with Ewing’s sarcoma can be cured. Even if the tumor comes back, additional treatment with surgery, radiation therapy, and/or chemotherapy may be successful. Surgery and radiation therapy can stop bones from growing and may result in asymetry. As a result, some children may require corrective surgeries later in life. Most patients successfully treated for Ewing’s sarcoma grow up with little disability.