Ewings Sarcoma Ewing's sarcoma is a cancerous (malignant) tumor that usually begins growing in a bone.

It occurs primarily in children and young adults, often appearing during the teen years. Although Ewing's sarcoma can develop in any bone, it usually affects the long bones, such as the thighbone (femur), shinbone (tibia), and upper arm bone (humerus). The bones of the pelvis are also often affected. Occasionally, the tumor begins in the muscles and soft tissues (this is called extraosseous Ewing's sarcoma). Ewing's sarcoma can spread (metastasize) to other parts of the body, such as the lungs, bone marrow, and other soft tissues. When compared with other cancers, malignant bone tumors like Ewing's sarcoma are rare. Of these rare tumors, Ewing's sarcoma is the second most common in children and young adults. According to data on children younger than 15 years old, approximately 1.7 children out of a million develop the disease. Recently, doctors have defined the disease to include four types of cancer, referred to as the Ewing's Family of Tumor (EFT). This includes the Ewing's sarcoma of bone, the Ewing's sarcoma of soft tissue, primitive neuroectodermal tumor (PNET) which may occur in both bone and soft tissue, and Askin's tumor, a PNET that occurs in the bones of the chest.

Cause There is no known cause of Ewing's sarcoma. What is known is that the cancer forms when changes occur in a cell's chromosomes. In Ewing's sarcoma cells, the genetic material in chromosomes #11 and #22 is mismatched. This genetic abnormality is not inherited from a child's parents - the chromosomal changes occur after a child is born. It is not understood why this abnormality occurs. Doctors have not identified any risk factors that make one child more susceptible than another. The tumor does not develop as a result of any dietary, social, or behavioral habits. There are no known ways to prevent the disease, and parents should know that there is nothing they could have done differently to prevent their child's tumor.

Symptoms There is usually pain and possibly swelling at the site of the tumor. But, the tumor may be present for many months before it becomes large enough to cause pain and swelling. Although injuries are not a known cause, an injury may draw attention to a tumor. For example, a bone weakened by a tumor may break after a minor injury.

Diagnosis After discussing symptoms and conducting a physical examination, your doctor will use several types of tests to diagnose Ewing's sarcoma. 1) Imaging Tests First, a doctor uses imaging studies to create detailed pictures of the affected area. These include xrays, magnetic resonance imaging (MRI) scans, computed tomography (CT) scans, and bone scans.

This x-ray shows the knee of a 14-year old girl with Ewing's sarcoma. The tumor is toward the top of the fibula and has destroyed a portion of the bone.
Reproduced from Domson G, Scarborough M, Gibbs CP: Malignant bone tumors in children. Orthopaedic Knowledge Online 2007. Accessed September 2011.

2) Biopsy To confirm that the tumor seen in imaging studies is Ewing's sarcoma, your doctor will do a biopsy. This involves taking a piece of tissue from the tumor and looking at it under a microscope. A biopsy is a simple procedure that may be done in an operating room or x-ray department. Ewing's sarcoma tumors are sometimes called small blue cell (round cell) tumors because of the way they look under a microscope.

The small, round, blue cells of Ewing's sarcoma as seen under a microscope.
Reproduced from Domson G, Scarborough M, Gibbs CP: Malignant bone tumors in children. Orthopaedic Knowledge Online 2007. Accessed September 2011.

3) Staging Once the tumor is identified as Ewing's sarcoma, your doctor will conduct more tests to determine whether the cancer has spread. This process is known as "staging." These additional tests may include: Blood tests CT scan of the lungs Bone scan Bone marrow biopsy The part of the body where the first tumor develops is called the "primary" site. Any parts of the body where it has spread are called "metastatic" sites. By identifying the stage of the tumor, your doctor can determine the most effective treatment strategy.

Treatment At the time of diagnosis, many cases of Ewing's sarcoma have not spread to other parts of the body. Even if tests do not show spread, however, doctors will plan a treatment strategy that assumes a very small amount of spread (micrometastatic disease) has already happened. Doctors in many specialties help treat Ewing's sarcoma. These include orthopaedic surgical oncologists, pediatric or adult medical oncologists, radiation oncologists, pathologists, and radiologists. Most patients are treated at major hospital institutions or cancer centers. The main treatments are chemotherapy, surgery, and radiation. These treatments are often used in some combination with each other. Both surgery and radiation are effective treatments for removing the primary tumor. In most cases, doctors use surgery to remove the tumors when possible. Radiation treatment is used only when surgery cannot completely remove the tumor or would cause the patient to lose function in the affected area. 1) Chemotherapy Various medications are used in chemotherapy treatment for Ewing's sarcoma. The most common drugs are vincristine, dactinomycin, cyclophosphamide, and doxorubicin. Recent evidence shows the addition of ifosfamide and etoposide helps. 2) Surgery

Some Ewing's sarcoma tumors cannot safely be removed with surgery. In these cases, doctors typically use radiation therapy to destroy the tumor. Occasionally, a tumor requires amputation of the limb to ensure that the tumor and surrounding tissues have been completely removed. 3) Radiation When radiation treatment is used, daily treatments are given over the course of many weeks. While the discomfort of surgery may be avoided, there are risks associated with the radiation, including: Skin damage Muscle scarring and loss of joint flexibility Damage to nearby organs Loss of bone growth in growing children Secondary cancers caused by radiation Chronic swelling of an extremity Slow wound healing