Understanding a Rare, Deadly Cancer: Ewing Sarcoma

When a young person is given a prognosis of one year to live, everyone wants a cure to appear, as if by magic.

The good news is that the medical community is actively studying rare cancers like Ewing Sarcoma and searching for more effective, well-tolerated treatments. Researchers are exploring innovative approaches while doctors are helping cancer patients and their families balance quality of life with treatments designed to extend life.

Jonathan Trent, M.D., an oncologist specializing in the diagnosis and treatment of sarcomas at the Sylvester Comprehensive Cancer Center says that rare cancers can be difficult to prevent and treat because we’re still learning about them.

What is Ewing Sarcoma?

It is a tumor (or tumors) that can develop in a person’s bone or soft tissue at any age, but this cancer is one type that may be diagnosed during childhood and the early teen years.

Symptoms may begin with pain in the affected bone or surrounding soft tissue, which is typically in the head or neck, pelvis, arms and/or legs. Other signs include a soft, warm lump (the tumor), broken bones weakened by the tumor, fever, weight loss and physical exhaustion.

“While these symptoms could be the sign of something far less threatening than cancer, it’s recommended that parents and young adults pay attention to such indicators and seek a medical evaluation including x-rays, when appropriate,” says Dr. Trent.

After symptoms appear with an abnormality on the X-ray, a diagnosis can be confirmed with a biopsy performed by an experienced sarcoma specialist and reviewed by a sarcoma pathologist.

What’s the prognosis?

When this cancer is diagnosed and treated before it has spread to other organs, 70% of Ewing Sarcoma patients live for more than five years following treatment. Initially, chemotherapy is used to treat the tumor and halt the spread of cancer. After chemotherapy, surgery or radiation is used to treat the tumor at the initial site and sometimes at places it has spread.

Once the cancer has metastasized, the five-year survival rate is 30%. The survival rate is higher for those with cancer that spread to the lungs, as compared to other organs.

“While we are working to raise survival rates and improve quality of life for those living with Ewing Sarcoma, we want people to keep in mind this remains a very rare form of cancer requiring specialists to ensure correct diagnosis and therapy,” says Dr. Trent.

Is Ewing Sarcoma genetic?

Cancer can run in your family and put you and your children at a higher risk. But knowing this risk may lead to testing or screening to see if you carry certain genetic markers. “This is not the case with Ewing Sarcoma,” Dr. Trent says.

“Although we can identify the chromosome changes that trigger most Ewing Sarcomas, these changes are not passed down from generation to generation.

“We do know that this type of cancer is much more common among whites (either non-Hispanic or Hispanic) than among blacks or Asians, and is slightly more common in males than females. The reason for these differences isn’t yet understood.”

Can Ewing Sarcoma be prevented?

With certain cancers, your lifestyle choices, like tobacco, drinking alcohol, exercise, your weight, and diet, may contribute to or lessen your risk. “But these risk factors do not appear to initiate the onset of Ewing Sarcoma,” said Dr. Trent. “Unhealthy habits can take years to impact your cancer risk, and this type of cancer is known to develop quickly in young people.”

Additionally, Ewing tumors in children are not linked to environmental exposures such as radiation and chemicals.

“The medical community is working to better understand what causes the genetic changes that trigger Ewing’s Sarcoma development so we can better advise the public on prevention, early detection, optimal treatment and, hopefully, one day a cure.”

Story updated 7/29/20.
Dana Kantrowitz is a contributing writer for UMiami Health News.

Originally published on: February 25, 2019

Tags: Dr. Jonathan Trent, Ewing Sarcoma, pediatric oncology, soft tissue sarcoma