Ewing Sarcoma Causes and Symptoms

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, although not impossible, and it accounts for approximately 2% 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 extremity, as well as the pelvis.

The Ewing Sarcoma Family of Tumors encompasses a spectum of sarcomas that were once thought to be distinct diseases, but research evidence indicates that they are variants of the same condition. These tumors can occur within bone or soft tissues. 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. Without proper testing from a trained physician, Ewing sarcoma is difficult to diagnose. With early diagnosis prior to the development of metastatic sites of disease, high rates of survival can be expected with modern radiation, surgical techniques, and chemotherapy protocols.

Types of Ewing Sarcoma

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 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 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.

Causes of Ewing Sarcoma

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, researchers have been able to narrow down several key triggers:

  • Chromosomal changes to cells – We now know that Ewings sarcoma family of tumors is the result of specific genetic alterations within the tumor cells, which is responsible for its malignancy.

Symptoms of Ewing Sarcoma

There are a variety of symptoms that go along with Ewing sarcoma that sometimes make it difficult to detect right away. Because this form of bone tumor is so rare, many symptoms appear similar to other common bone diseases.

Here are some of the most common symptoms found in those people who suffer from Ewing sarcoma:

  • Pain or tenderness at the site of the tumor
  • Varying intensities of localized pain
  • Bone pain
  • Swelling
  • Fever
  • Broken bone

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 if you experience any of the above symptoms so you can be quickly diagnosed and on the path to recovery.

The multidisciplinary team of orthopaedic experts at North Shore-LIJ Orthopaedic Institute's musculoskeletal oncology services in New York treats Ewing Sarcoma as well as a broad range of conditions that affect the bones within the body.

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