Ewing Sarcoma in Adults

Ewing’s Sarcoma: Symptoms, Causes, and More

Ewing Sarcoma in Adults | Johns Hopkins Medicine

Ewing’s sarcoma is a rare cancerous tumor of bone or soft tissue. It occurs mostly in young people.

Overall, it affects 1 every 1 million Americans. But for adolescents aged 10 to 19 years, this jumps to about 10 cases per 1 million Americans in this age group.

This means that about 200 cases are diagnosed in the United States each year.

The sarcoma is named for the American doctor James Ewing, who first described the tumor in 1921. It isn’t clear what causes Ewing’s, so there are no known methods of prevention. The condition is treatable, and, if caught early, full recovery is possible.

Keep reading to learn more.

The most common symptom of Ewing’s sarcoma is pain or swelling in the area of the tumor.

Some people may develop a visible lump on the surface of their skin. The affected area may also be warm to the touch.

Other symptoms include:

  • loss of appetite
  • fever
  • weight loss
  • fatigue
  • general unwell feeling (malaise)
  • a bone that breaks without a known reason
  • anemia

Tumors typically form in the arms, legs, pelvis, or chest. There may be symptoms specific to the location of the tumor. For example, you may experience shortness of breath if the tumor is located in your chest.

The exact cause of Ewing’s sarcoma isn’t clear. It isn’t inherited, but it can be related to non-inherited changes in specific genes that happen during a person’s lifetime. When chromosomes 11 and 12 exchange genetic material, it activates an overgrowth of cells. This may lead to the development of Ewing’s sarcoma.

Research to determine the particular type of cell in which Ewing’s sarcoma originates is ongoing.

Although Ewing’s sarcoma can develop at any age, more than 50 percent of people with the condition are diagnosed in adolescence. The median age of those affected is 15.

In the United States, Ewing’s sarcoma is nine times more ly to develop in Caucasians than in African-Americans. The American Cancer Society reports that the cancer rarely affects other racial groups.

Males may also be more ly to develop the condition. In a study of 1,426 people affected by Ewing’s, 59 percent were male and 41 percent were female.

If you or your child experience symptoms, see your doctor. In about 25 percent of cases, the disease has already spread, or metastasized, by the time of diagnosis. The sooner a diagnosis is made, the more effective treatment may be.

Your doctor will use a combination of the following diagnostic tests.

Imaging tests

This may include one or more of the following:

  • X-rays to image your bones and identify the presence of a tumor
  • MRI scan to image soft tissue, organs, muscles, and other structures and show details of a tumor or other abnormalities
  • CT scan to image cross-sections of bones and tissues
  • EOS imaging to show the interaction of joints and muscles while you’re standing
  • bone scan of your whole body to show if a tumor has metastasized
  • PET scan to show whether any abnormal areas seen in other scans are tumors


Once a tumor has been imaged, your doctor can order a biopsy to look at a piece of the tumor under a microscope for specific identification.

If the tumor is small, your surgeon may remove the whole thing as part of the biopsy. This is called an excisional biopsy, and it’s done under general anesthesia.

If the tumor is larger, your surgeon may cut away a piece of it. This may be done by cutting through your skin to remove a piece of the tumor. Or your surgeon may insert a large, hollow needle into your skin to remove a piece of the tumor. These are called incisional biopsies and are usually done under general anesthesia.

Your surgeon may also insert a needle into the bone to take out a sample of fluid and cells to see if the cancer has spread into your bone marrow.

Once the tumor tissue is removed, there are several tests that help identify an Ewing’s sarcoma. Blood tests may also contribute helpful information for treatment.

Types of Ewing’s sarcoma

Ewing’s sarcoma is categorized by whether the cancer has spread from the bone or soft tissue in which it started. There are three types:

  • Localized Ewing’s sarcoma: The cancer hasn’t spread to other areas of the body.
  • Metastatic Ewing’s sarcoma: The cancer has spread to the lungs or other places in the body.
  • Recurrent Ewing’s sarcoma: The cancer doesn’t respond to treatment or returns after a successful course of treatment. It most often recurs in the lungs.

Treatment for Ewing’s sarcoma depends on where the tumor originates, the size of the tumor, and whether the cancer has spread.

Typically, treatment involves one or more approaches, including:

Treatment options for localized Ewing’s sarcoma

The common approach for a cancer that hasn’t spread is a combination of:

  • surgery to remove the tumor
  • radiation to the tumor area to kill any remaining cancer cells
  • chemotherapy to kill possible cancer cells that have spread, or micrometastasies

Researchers in one 2004 study found that combination therapy such as this was successful. They discovered the treatment resulted in a 5-year survival rate of approximately 89 percent and an 8-year survival rate of about 82 percent.

Depending on the tumor site, further treatment may be necessary after surgery to replace or restore limb function.

Treatment options for metastasized and recurrent Ewing’s sarcoma

Treatment for Ewing’s sarcoma that has metastasized from the original site is similar to that for the localized disease, but with a much lower success rate. Researchers in one 2010 study reported that the 5-year survival rate after treatment for metastasized Ewing’s sarcoma was about 70 percent.

There is no standard treatment for recurrent Ewing’s sarcoma. Treatment options vary depending on where the cancer returned and what the previous treatment was.

Many clinical trials and research studies are ongoing to improve treatment for metastasized and recurrent Ewing’s sarcoma. These include:

  • stem cell transplants
  • immunotherapy
  • targeted therapy with monoclonal antibodies
  • new drug combinations

As new treatments develop, the outlook for people affected by Ewing’s sarcoma continues to improve. Your doctor is your best resource for information about your individual outlook and life expectancy.

The American Cancer Society reports that the 5-year survival rate for people who have localized tumors is about 70 percent.

For those with metastasized tumors, the 5-year survival rate is 15 to 30 percent. Your outlook may be more favorable if the cancer hasn’t spread to organs other than the lungs.

The 5-year survival rate for people with recurrent Ewing’s sarcoma is 10 to 15 percent.

There are many factors that may affect your individual outlook, including:

  • age when diagnosed
  • tumor size
  • tumor location
  • how well your tumor responds to chemotherapy
  • blood cholesterol levels
  • previous treatment for a different cancer
  • gender

You can expect to be monitored during and after treatment. Your doctor will periodically retest to determine whether the cancer has spread.

People who have Ewing’s sarcoma may have a higher risk of developing a second kind of cancer. The American Cancer Society notes that as more young people with Ewing’s sarcoma are surviving to adulthood, the long-term effects of their cancer treatment may become apparent. Research in this area is ongoing.

Source: https://www.healthline.com/health/ewings-sarcoma

Affordable Care Act gives lifeline for young patient

Ewing Sarcoma in Adults | Johns Hopkins Medicine

At Hopkins, Jen Edwards recalls, hospital administrators made a crucial discovery: Ella had been admitted through the emergency room.  If Ella was discharged, Johns Hopkins would not readmit her because, though the emergency visit was covered, Hopkins did not accept her insurance for continuing treatment, a staff member confirmed.

They stopped the family from leaving.  The administrators recommended that Brian Edwards purchase a new plan, under “Obamacare,” that would cover Ella’s future treatment — avoiding a bill of $80,000.  

In a stroke of luck, Hague Quality Water was in a two-week period where the business could choose a new insurance provider for their employees. Brian Edwards switched his company’s coverage to Evergreen Health, a plan on the state health exchange that offered in-state health insurance for Ella’s condition.

Jen and Brian Edwards sort through bills for Ella’s treatment. To date, the family’s insurance plan through an Affordable Care Act provider has absorbed over $200,000 in medical costs.

Ella’s newly diagnosed cancer is included on a list of declinable conditions that would have caused her application for insurance to be automatically denied in all but five states before the health care law, according to a study by the Kaiser Family Foundation.

Evergreen Health’s monthly premium is $1,900, nearly 30 times the $66 premium he previously paid for insurance covering all his children — the policy from a company that Johns Hopkins would not accept.

“Even if you can’t pay the bills in that moment, you’re still going to do the treatment,” Jen Edwards said.

She leafed through a thick, worn binder filled with letters from doctors, scraps of paper with hastily jotted notes, and bills — dozens of bills.

Ella’s initial seven-day hospitalization topped $41,000, including $17,000 for room and board, and $20,000 for her first round of chemotherapy.

A statement from Evergreen Health, the family’s insurance provider, details the cost of Ella’s initial hospital stay and treatment in April. Her first round of chemotherapy cost more than $20,000.

Four months of cancer treatments, visits with specialists, and hospitalizations racked up over $200,000. All but their $1,500 deductible was paid by their insurance company.

Before the Obama health care law, those costs led many families to bankruptcy.

A study conducted by Harvard University and published in the American Journal of Medicine in 2007 found that from 2001 to 2007, bankruptcies attributable to medical problems increased by 50 percent and comprised 67 percent of all bankruptcies in the United States.

Cost of life, a metric used to quantify one year of life with cancer treatment, rose from $54,100 in 1995, to $207,000 in 2013. This statistic does not include expenses surgery or home care, nor does it account for the loss of income resulting from a chronic illness.

Brian and Jen Edwards held a different view of the health care law before Ella’s diagnosis. Back then, they viewed “Obamacare” as socialization of health care.

“For me, Ella’s cancer changed my perspective about the Affordable Care Act,” Jen Edwards said.

“Knowing some of these children that are also at Hopkins, I know their families can’t afford it,” she trails off. “Every child should get care.”

Jen Edwards has quit her job at a local church to care for Ella.

Jen Edwards and her daughter Ella play cards to pass the time during Ella’s chemotherapy treatments. Each treatment session is eight hours long, and Ella has completed 11 sessions since her diagnosis in April.

Brian Edwards supplements his work-provided policy with an additional policy to cover the more expensive drugs not covered by Evergreen.

The additional policy is income-based. With five children and a single income, the Edwards family qualifies for its insurance. But if Jen Edwards were to resume working and the family income increased, they would be ineligible.

But even with government subsidies, the Edwards family’s health insurance policies cost him over $2,500 a month.

“It’s overwhelming,” Brian Edwards said. “I don’t know how people do it without insurance.”

Ewing’s sarcoma has a good prognosis if it has not spread. Ella’s has spread to her lungs.

Source: https://cnsmaryland.org/affordable-care-act-young-cancer-patient/

Ewing Sarcoma – NORD (National Organization for Rare Disorders)

Ewing Sarcoma in Adults | Johns Hopkins Medicine


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