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The University of Chicago Medicine - Comer Children's Hospital

Ewing Sarcoma

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.

What are the Symptoms 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.

State-of-the Art Diagnostics

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 procedures.

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.

Advanced Treatments

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 (like amputation) -- 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, like 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 very 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.

Innovative Surgery Saves Limbs, Protects Growth

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 prosthesis 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 worldwide performing "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.