Ewing sarcoma (also known as Ewing’s sarcoma) is a tumor that arises primarily in the bone and soft tissue. It can either affect the long bones such as the femur (thigh), tibia (shin) or humerus (upper arm), or the flat bones like the ribs, pelvis or spine. It also can involve the soft tissues around the tumor site.

While rare, Ewing sarcoma is the second most common malignant bone tumor in children and adolescents after osteosarcoma. It is slightly more common in males than females; and usually occurs in children and young adults between the ages of 10 and 20. Ewing sarcoma is highly malignant and can metastasize (spread) rapidly to other areas of the body including the bone marrow, lungs, kidneys, heart, adrenal gland and other soft tissues. Accurate diagnosis and staging, and prompt, carefully orchestrated therapy are particularly critical when treating Ewing sarcoma.

The University of Chicago Medicine Comer Children’s Hospital has a multidisciplinary team of experts with the experience and advanced resources to diagnose, analyze and treat Ewing and other bone sarcomas. Pediatric oncologists, radiation oncologists, orthopaedic surgeons, pathologists, nurse specialists and others at Comer Children’s work together to strive for the best results for children with bone sarcomas.

Symptoms, Diagnosis & Treatment of Ewing Sarcoma

The most common symptoms of Ewing sarcoma are:

  • Pain around the site of the tumor
  • Swelling and/or redness around the site of the tumor
  • Fever
  • Weight loss, decreased appetite
  • Fatigue
  • Paralysis and/or incontinence (if the tumor is in the spinal region)
  • Symptoms related to nerve compression from the tumor (e.g., numbness, tingling, paralysis, etc.)

These symptoms may resemble other conditions or medical problems seen in children and young adults. Always consult a physician for a diagnosis.

Getting the correct diagnosis is critical, but isn’t always simple. Multilevel, advanced diagnostics are necessary to detect, locate, identify and stage Ewing sarcoma. Once a tumor is suspected, your care team will perform imaging studies and procedures including:

  • X-ray to confirm the presence of a tumor
  • Magnetic resonance imaging (MRI) or computed tomography (CT) scan to determine the extent of the tumor
  • Bone scan to identify any additional sites of bone involvement
  • Positron emission tomography (PET) scan to find small tumors or check if treatment is working effectively
  • Biopsy to remove tissue from the tumor for microscopic examination by an expert pathologist. Because Ewing sarcoma can also spread to the bone marrow, a sample of bone marrow is also collected as part of the staging process.

Using extensive, specialized testing of the tissue — often including molecular tests for genetic abnormalities within the Ewing Sarcoma — the pathologist is able to confirm the diagnosis and identify the type of sarcoma. The sarcoma team then assigns a stage to the tumor, defining whether the tumor is localized to only one site or has spread to other sites. Having the correct diagnosis and stage assignment is critical for optimal treatment planning.

The treatment of Ewing sarcoma occurs in several phases aimed at both treating the primary tumor that is visible and treating tumor cells that have spread around the body, even if those tumor cells are not detectable by routine tests. If the primary tumor can be safely removed with surgery, this is often the first step in the treatment. If the primary tumor cannot be safely removed — because, for example, it is too big or is located in a site making it impossible to remove without serious complications — chemotherapy is often used first to shrink the tumor before surgery.

In some cases, the primary tumor simply cannot be removed because it involves critical structures, such as bones in the spine or the pelvis. In these cases, radiation therapy to control the primary tumor is often used in place of surgery and is a very effective way to control the primary tumor. After the primary tumor is surgically removed (or treated by radiation), additional chemotherapy is given to destroy any remaining cancer cells at the primary site and in other sites. This is very important because we know that most patients with Ewing sarcoma have microscopic amounts of tumor cells spreading around the body. Without effective chemotherapy, these cells form into recurrent or relapsed tumors at new sites in the body. In some specialized settings, very high doses of chemotherapy are used in association with a stem cell (or bone marrow) transplant to eradicate any remaining Ewing sarcoma cells.

At Comer Children's, we have the capability to provide all of these treatment options on-site, which enables the team to effectively coordinate each child's care and to readily modify treatment plans as the cancer responds to therapy.

Some patients may be eligible for studies of the newest therapies through clinical trials being conducted at Comer Children's. Parents are closely involved in deciding whether or not to enroll their child in a clinical trial. Clinical trials are only considered by the sarcoma team if they are thought to provide the best chance to cure the Ewing sarcoma.

We carefully balance the need to surgically treat bone cancer with the desire to keep kids active and functioning normally throughout their childhood and into adulthood. Different types of surgery may be required during treatment for Ewing sarcoma. Surgeries are tailored to fit the needs of each patient:

  • Resection: Removal of the original tumor
  • Limb-sparing surgery: Removal of the affected bone, saving the limb from amputation. This is done in most cases.
  • Articular-sparing surgery: Removal of the tumor and reconstructing the bone/limb while preserving the joints
  • Amputations: Removal of all or part of the limb. It is used as a last resort, when it is the only option for cure.
  • Metastasis surgery: Removal of tumor that has spread to other sites, such as the lungs

Orthopaedic surgeons at Comer Children's have been leaders in less-extreme surgical treatment of bone cancers for more than 20 years. In the 1980s, our surgeons were among the first in the U.S. to perform "limb-sparing" surgery as an alternative to amputation. We use state-of-the-art prostheses to rebuild the involved cancerous bone and joint removed during surgery. With physical therapy, nearly all patients are able to walk soon after surgery.

Today, our surgeons are among the first in the world to perform "articular-sparing” (joint-sparing) resection to preserve the joint surfaces and long-term functioning for kids with bone cancer. This intricate technique preserves as much of the child's healthy bone as possible. On certain scenarios, we are able to also spare the growth plates to preserve bone growth as the child grows. Like matching together jagged pieces of a jigsaw puzzle, the orthopaedic surgeon removes cancerous bone and then fits together the child's own healthy bone with bone from a bone tissue bank (i.e. allograft bone). The result: a bone or joint that is strong, stable and has the best chance to grow normally.

Frequently Asked Questions About Ewing Sarcoma

What causes Ewing sarcoma?

Exactly what triggers Ewing sarcoma is not known. But we do know that the cancer cells in Ewing sarcoma almost always have a specific chromosomal abnormality causing normal cells to grow out of control. It is important to know that this chromosomal abnormality is not something a child inherits; it seems to be an "accident" that occurs in a cell sometime after birth. Often, the sarcoma cells are tested for this particular chromosomal abnormality, providing a tool that confirms the diagnosis of Ewing sarcoma.

What are the risk factors for Ewing sarcoma?

There are no known risk factors for Ewing sarcoma and no known way to prevent the disease.

Do I have to worry that my other children will get Ewing sarcoma?

It’s rare for other family members to be affected. We always analyze family history for each new patient. Occasionally a genetic problem is suspected. In that case, the patient and certain family members are sometimes referred to our Pediatric Familial Cancer Center to be tested for inherited defects that might predispose them to cancer.

What is staging?

Once the diagnosis has been confirmed, your oncologist will assign a stage to the tumor. Ewing sarcomas are staged as either localized or metastatic (spread to other parts of the body).

Where does Ewing sarcoma usually spread?

If the tumor cells have metastasized (spread), they can be found in other areas of the body, including the bone marrow, lungs, kidneys, heart, adrenal gland and other soft tissues.

Will my child need surgery?

Ewing sarcoma treatment often includes surgery for the initial biopsy and removal of the primary tumor that is visible. When the tumor occurs in a bone, this is usually done with bone grafts, limb-sparing procedures and reconstruction. In cases where the primary tumor cannot be completely removed, radiation therapy is often used with, or in place of, surgery. Because radiation treatment is very effective, amputation is rarely necessary for Ewing sarcoma.

Will my child need chemotherapy? If so, how is it given?

Chemotherapy, before and after surgery, is a critical part of the comprehensive and multidimensional treatment of Ewing sarcoma. It is sometimes given before surgery in order to shrink the tumor, which can make surgical removal easier to accomplish. Chemotherapy is also given after surgery, even if there is no other evidence of tumor left behind. This is done because we know that microscope amounts of Ewing sarcoma remain after surgery. Chemotherapy is used to eradicate these cells to minimize the chance that the cancer will come back at other sites. Chemotherapy for Ewing sarcoma is nearly always given intravenously (IV) through a catheter — called a "central line." Chemotherapy is usually given over a period of two to five days in the hospital. Each of these cycles of chemotherapy is given every two to three weeks. Usually, about 14 cycles of chemotherapy are used.

Will my child need radiation treatment?

Radiation is often given to sites where the Ewing sarcoma is visible. In situations where surgery cannot be used, radiation treatments can effectively control the tumor and prevent the cancer from growing. In some cases, radiation therapy is used after a primary tumor is removed in an effort to get rid of any cancer cells that might have been on the margin or edge of the tumor removal site.

How long will treatment take? How long will my child be in the hospital?

Chemotherapy for Ewing sarcoma is usually given in cycles, with each cycle covering a period of two to five days in the hospital. A new cycle is given every two to three weeks. Usually, about 14 cycles of chemotherapy are needed. The total duration can be from 30 to 45 weeks. At some stage during the treatment, surgery is often performed to remove the visible tumor. The recovery period in the hospital varies from patient to patient, lasting from several days to one to two weeks. Radiation therapy, if needed, is given five days per week for four to six weeks. It is administered in the outpatient treatment center. Chemotherapy is often modified or delayed during surgery or radiation therapy.

What are the side effects of treatment?

Chemotherapy drugs not only attack the cancer cells, they can injure normal tissue and blood-forming cells. For this reason, your child may experience some of the common side effects of chemotherapy including low blood counts (causing anemia), nausea, vomiting, hair loss, mouth sores, irregular periods (girls) and inability to produce sperm (boys). Radiation therapy may put children at a risk of developing a second malignant tumor later in life. Your treatment team will do everything they can to reduce or prevent side effects of treatment.

Does treatment cause infertility?

Patients and their parents should talk to their doctor about the reproductive risks associated with cancer treatment. In certain situations, there are options available to try to preserve fertility, such as with sperm banking. These programs are available at Comer Children’s.

What is the prognosis for survival?

The prognosis for survival varies depending on the stage of the tumor when it is detected and treated and other factors. The survival rate for Ewing sarcoma patients who undergo chemotherapy and surgery, and who have no metastases, is 60 to 70 percent. About 20 to 25 percent of children with metastatic Ewing sarcoma are cured of the disease.

Can Ewing sarcoma come back?

Unfortunately, Ewing sarcoma can come back after treatment. If Ewing sarcoma comes back, it can often be treated with surgery, additional radiation therapy and additional chemotherapy, including more experimental medicines and (possibly) stem cell transplant.