Ewing sarcoma is a rare primary bone sarcoma, most commonly affecting adolescents and young adults between the ages of 10 and 20. Ewing sarcoma is rare among adults. It accounts for approximately two percent of all childhood cancer cases, affecting approximately 250 children per year in the United States. Males are also more susceptible to this form of cancer at a ratio of almost 1.6 to 1. When confined to bone, Ewing sarcoma most commonly occurs in the long bones of the lower and upper extremities, as well as the pelvis.
The Ewing sarcoma family of tumors encompasses a spectrum of sarcomas that once were thought to be distinct diseases, but research indicates that they are variants of the same condition. These tumors can occur within bone or soft tissues.
There is little known about what causes Ewing sarcoma, and because the tumors form at such a young age, there is no known way to prevent the disease from striking. However, we now know that the Ewing sarcoma family of tumors is the result of specific genetic alterations within the tumor cells, which is responsible for its malignancy. Despite having a defined genetic alteration that is responsible for tumor development, Ewing sarcoma is inherited. It is caused by a shift between specific chromosomes.
Because Ewing sarcoma symptoms are very similar to those of other diseases, it is easy to misdiagnose or difficult to determine right away, especially since this form of bone tumor is so rare. Here are some of the most common symptoms found in those who suffer from Ewing sarcoma:
- Pain or tenderness at the site of the tumor
- Varying intensities of localized pain
- Bone pain
- Broken bone
Ewing sarcoma is often referred to as the Ewing sarcoma family of tumors, which includes:
- Ewing sarcoma of bone – This was the form of Ewing sarcoma originally discovered by Dr. James Ewing. It consists of a single type of small round cell.
- Primitive neuroectodermal tumor (PNET) – Originally felt to be distinct from Ewing sarcoma, this tumor is now known to be part of the same malignant process. The cells are slightly better differentiated than those of Ewing sarcoma. Some clinicians use this terminology to describe tumors in soft tissue, rather than in bone.
- Askin tumor – The Askin tumor has the same cellular makeup of the other forms of Ewing sarcoma, but is used to describe its location in the chest wall.
Ewing sarcoma symptoms are very similar to those of other diseases, and so the disease can easily be misdiagnosed or difficult to determine right away. For this reason it is important to see a trained doctor for proper testing if you experience any of the above symptoms, so you can be quickly diagnosed and on the path to recovery. With early diagnosis prior to the development of metastatic sites of disease, high rates of survival can be expected through use of modern radiation, surgical techniques and chemotherapy protocols.
The multidisciplinary team of musculoskeletal oncology experts at Northwell Health Orthopaedic Institute treats Ewing sarcoma as well as a broad range of conditions that affect the bones.