Ewing Sarcoma | Diagnosis & Treatment

How is Ewing sarcoma diagnosed?

The first step in treating your child is forming an accurate and complete diagnosis. Ewing sarcoma is typically diagnosed using a combination of imaging and biopsy. Your child’s physician may order a number of different tests including:

  • a physical exam and complete medical history
  • magnetic resonance imaging (MRI), a diagnostic procedure that produces detailed images. An MRI uses a combination of large magnets, radiofrequencies, and a computer to analyze organs and structures within the body
  • CT/CAT scan, an imaging technique that provides more detailed pictures than X-rays
  • positron emission tomography (PET) scan, a test commonly used to detect cancers and help doctors see how the organs and tissues inside the body are functioning
  • bone scan, used to detect bone diseases and tumors, as well as to determine the cause of bone pain or inflammation
  • bone marrow aspiration and biopsy, a procedure that involves taking a small amount of bone marrow fluid and tissue (usually from part of both hip bones) to look for signs of Ewing sarcoma cells
  • tumor biopsy, a procedure that can involve a surgeon removing a small piece of the tumor or a specialized radiologist using a needle to obtain a part of the tumor

There may be other diagnostic tests that your doctor will discuss with you depending on your child's individual situation. After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best possible treatment options.

What are the treatment options for Ewing sarcoma?

Most of the time, there are two components in treating children with newly diagnosed Ewing sarcoma: local control, which involves treating the tumor itself, usually through surgery, radiation, or both; and systemic therapy, which treats any tumor cells throughout the body, usually through chemotherapy. Ewing sarcoma can usually only be cured by using local control together with systemic therapy. Your child's treatment may include (alone or in combination):

Chemotherapy is usually given over a period of a few days every two weeks for about 12 weeks before it is time for local control of the main tumor. After surgery and/or radiation for local control, chemotherapy is continued for another four to six months in order to eliminate all the cancerous cells in the body. We give chemotherapy cycles every two weeks in Ewing sarcoma because studies show that this schedule (called “interval compression”) improves outcomes for children with localized Ewing sarcoma.

The typical chemotherapy regimen for a child with newly diagnosed Ewing sarcoma involves medicines given intravenously as a direct injection into the blood stream. This chemotherapy used to be given during overnight stays in the hospital. Now through our home hydration program, most children are able to receive this regimen as an outpatient.

While chemotherapy can be quite effective in treating certain cancers, the drugs cannot differentiate normal healthy cells from cancer cells. As a result, there can be adverse side effects during treatment. Being able to anticipate these side effects can help the care team, child, and family prepare (and, in some cases, prevent) these complications from occurring, if at all possible.

Surgery

Local control of Ewing sarcoma is achieved with surgery and/or radiation therapy. Surgery may be performed after the first 12 weeks of chemotherapy to remove any parts of the tumor that remain. Several forms of surgical intervention may be considered for your child depending on the size and location of the tumor.

Limb-salvage surgery

Limb-salvage surgery helps preserve the limb by removing the tumor and wide margins of healthy tissue surrounding the tumor. The goal of limb-salvage surgery is to preserve limb function, as well as the pre-surgical appearance of the limb. However, limb-salvage surgery can leave an arm or leg fragile and increase the risk of fracture. As a result, patients will need to avoid high-stress physical activities, such as skiing, skateboarding, or bike riding.

Amputation

Amputation may be necessary if the tumor cannot be completely removed (for example, if it involves the nerves and blood vessels) or if limb function cannot be preserved through limb-salvage surgery. If amputation is necessary, your child may be fitted for a prosthesis following surgery.

Rotationplasty

Rotationplasty is a partial amputation that preserves a cancer-free lower leg, attaches it to the thighbone, and uses the ankle as a knee joint – especially useful in very young children where limb length can be an issue. One of the major benefits of rotationplasty compared to other surgical options is that it allows the child to maintain a very active lifestyle, including high-impact sports.

Limb-salvage surgery and rotationplasty are complex procedures that require specialized expertise. Our bone tumor surgeons are among the few orthopedic surgeons in the nation trained in pediatric tumor surgery and who have experience in these procedures. At Dana-Farber/Boston Children's, we also have physical therapists and prostheticians who specialize in helping children who undergo these procedures.

What is the long-term outlook for children with Ewing sarcoma?

For patients with localized Ewing tumors, the five-year survival rate is close to 70 to 80 percent. For people with metastatic disease, the five-year survival rate is 20 to 30 percent.

Patients who have Ewing sarcoma that has come back after initial treatment (called “relapsed” or “recurrent” disease) have a poor prognosis. Children whose disease recurs more than two years after initial diagnosis have a five-year survival rate of 30 percent, while very few patients whose disease recurs less than two years from initial diagnosis will survive with our current best therapies.

However, it’s important to understand that your child’s short-term health and long-term health can vary greatly depending on his specific circumstances. Prompt medical attention and intensive therapy are very important, as is continuous follow-up care.

Long-term care for Ewing sarcoma

Because Ewing sarcoma does have a tendency to recur later in life — even as many as 10 years after treatment — long-term follow-up care is essential. We see patients with Ewing sarcoma for X-rays or MRI of the primary tumor site and CT scan of the chest every three months after treatment is completed. If all goes well, the frequency of the visits will decrease for the next five years.

Some children who are treated for Ewing sarcoma and other pediatric cancers can experience significant long-term problems as a result of their treatment. All kids who have been treated for cancer require ongoing, specialized cancer survivorship care.

Through the David B. Perini Jr. Quality of Life Clinic, our cancer survivorship clinic, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team. In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists, or alternative/complementary therapy specialists. We also offer patient and family education, psychosocial assessment, genetic counseling, reproductive counseling, and opportunities to speak with other childhood cancer survivors.