Patients' Guide to Spinal Cancer

Ewing or Ewing’s Sarcoma is a Type of Spinal Cancer

Third most common type of primary bone cancer

Ewing sarcoma, by some termed Ewing’s sarcoma, is a malignant tumor most commonly found in long bones—and it can form in the spine’s bones. This cancer is the third most common primary bone cancer, and second most common bone cancer among children, teens, and young adults. Teens and young adults are at greatest risk of developing Ewing sarcoma.

teenage boys and girls lying in the grass

As it relates to the spine, Ewing sarcoma is a type of primary spinal tumor. Primary means that a tumor is at the site where it originated, as opposed to a tumor that developed elsewhere in your body then metastasized, or spread, to a different location.

Ewing sarcoma is the most common member of the Ewing sarcoma family of tumors, which also includes extraosseous Ewing tumor (affects soft tissues around bone, but behaves like Ewing sarcoma in bone) and peripheral primitive neuroectodermal tumor (rare childhood cancer often starting in the chest wall). This article will focus exclusively on Ewing sarcoma and how it affects the spine.

What Is Ewing Sarcoma?
Ewing sarcoma gets its namesake from Dr. James Ewing, who first described Ewing sarcoma in 1921, when he identified differences between it and another more common bone tumor called osteosarcoma.

Ewing sarcoma typically occurs in bones throughout the body, including the pelvis and the spine. Less commonly, the disease can originate in other tissues and organs. When Ewing sarcoma develops within the bones of your spine, you will likely experience significant back pain, in addition to other spine-related symptoms that you may learn more about below.

Like most primary spinal tumors, Ewing sarcoma is a rare disease, with fewer than 1,000 cases reported in the United States each year. It is most often present in adolescents and young adults—nearly half of cases occur between the ages of 10 and 20. Males are slightly more prone to Ewing sarcoma than females. Adults over age 30 may develop the disease, though it is extremely rare.

What Causes Ewing Sarcoma?
Scientists haven’t yet determined the exact cause of Ewing sarcoma, though research into gene changes that occur within the cancerous cells has shed light on the disease.

The tumor cells in Ewing sarcoma have a chromosomal mutation, and researchers believe this mutation plays a role in the disease. Most cases of Ewing’s sarcoma have a translocation between chromosome 11 and 22 that fuses the EWS gene to the FLI1 gene between the two chromosomes.  It’s important to understand that the chromosomal mutation is only apparent in the cancerous tumor cells—the mutation does not exist in the entire genome of the person with the disease. The change in the chromosome appears to develop sporadically in a cell after the person with Ewing sarcoma is born, but scientists don’t know why that happens.

These chromosomal events could be completely random, and Ewing sarcoma tumors are not caused by anything related to lifestyle or the environment. So, while much work is being done to better understand this disease, there’s no defined way to prevent it at this time.

Diagnosing Ewing Sarcoma
Your doctor may use a variety of tests and procedures to confirm a Ewing sarcoma diagnosis. After taking your full medical history and performing a physical exam, you may undergo imaging tests, such as CT scan or MRI to find the location of the tumor.

As Ewing sarcoma is a primary bone cancer, your doctor will likely perform a bone scan, and a bone marrow aspiration and biopsy to determine the extent of the cancer, and if it has spread.

A biopsy is essential to diagnose Ewing sarcoma. During a biopsy—which may be done using a needle or incision in the skin—tissue samples are removed and then examined for signs of cancer.

Spinal Symptoms of Ewing or Ewing’s Sarcoma
If Ewing sarcoma develops in the bones of your spine or pelvis, you may feel an array of symptoms that are localized in your back. Most people report back bone and joint pain, and you may have swelling and tenderness in your back near the site of the tumor. You may also feel a soft and warm lump at the tumor site. People who have a tumor in their pelvis with nerve compression of the sciatic nerve or lumbosacral plexus may experience shooting pain and other symptoms of sciatica.

Among the most serious symptoms of Ewing sarcoma is fracture, and some people have reported a broken bone without a clear cause (such as a fall).

Other symptoms associated with Ewing sarcoma include fever, discomfort, fatigue, appetite loss, and weight loss. Your team of doctors will discuss with you the best way to manage these symptoms to improve your comfort.

To learn about Dr. Hsieh's practice, click here.

Updated on: 02/02/17
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