What is Ewing sarcoma?

Ewing sarcoma is the second most common malignant (cancerous) bone tumor affecting children, adolescents and young adults, but it is still a rare disorder.

Ewing sarcoma is a cancer, which means it can spread to other organs or tissues in the body, most commonly the lungs.

Most Ewing sarcoma tumors are found in teenagers, but the disease can also occur at a younger age.

Treatment for Ewing sarcoma

There are many treatment options available for bone and soft tissue tumors, and some children will need a combination of these therapies. At Children’s Hospital of Philadelphia (CHOP), experts from the Bone and Soft Tissue Tumor Program take a team approach to treatment. Orthopedists, oncologists and other specialists collaborate to provide your child with individualized care and the best possible outcomes.

Chemotherapy

Treatment for Ewing sarcoma always involves adjuvant chemotherapy to target the primary tumor and cancer cells that may have already spread, but have not yet been detected. Chemotherapy refers to medicines that help fight cancer.

In most cases, chemotherapy is given to your child through an implantable venous port in his chest. The port remains in place for the duration of therapy to help your child avoid multiple needle sticks.

Surgery

Surgery for Ewing sarcoma has two goals:

• To remove the tumor
• To restore function at the site of the tumor

About 90 percent of children with Ewing sarcoma can be treated with limb-sparing (also known as limb-salvage) and reconstructive surgery. CHOP surgeons regularly perform these complex surgeries and are constantly working to improve outcomes for children with the most difficult-to-treat tumors.

Limb-sparing surgery is performed under general anesthesia. It involves cutting out the tumor with a margin of healthy tissue surrounding it. Depending on the size and location of the tumor, as well as your child’s age and stage of growth, surgeons may use a variety of reconstructive methods to restore your child’s body function.

Reconstruction may include:

Metal prosthesis:

• Metal prosthesis can be used to reconstruct the bone defect created by resection of the tumor, or by replacing the joint affected by the tumor. Joint replacement may be used if your child’s Ewing sarcoma is located on or near his knee, hip or shoulder. If your child is still growing, a modified joint replacement that can be expanded as your child grows, may be used.

Bone:

• The defect may be reconstructed with either the patient’s own bone such as vascularized fibula, or allograft (cadaver bone). Allograft (cadaver bone) if your child’s tumor is in the middle of an arm or leg bone. This replacement bone serves as a structural filler — giving your child’s limb more strength and durability as his own bone grows around it.
• Free vascularized fibular grafting if your child’s tumor is in the thigh bone. This procedure includes moving one bone from your child’s lower leg to replace a diseased thigh bone. This may be done along or in conjunction with an allograft.

In about 10 to 20 percent of cases — because of the size or location of the tumor — Ewing sarcoma cannot be removed with limb-sparing surgery. In these situations, two surgical options exist:

• Amputation of the affected limb.
• Functional amputation (rotationplasty). Children’s Hospital of Philadelphia is one of very few hospitals offering this complex surgery in which the ankle assumes the function of a knee and a prosthesis is attached to the foot. Children can resume active lifestyles and have a near normal function.

After surgery for Ewing sarcoma, your child should expect to stay two to five days in the Hospital.

Radiation therapy

Depending on your child’s individual situation, radiation therapy may be an option to treat Ewing sarcoma. Radiation therapy uses high-energy waves such as X-rays to kill or shrink cancer cells. Children’s Hospital of Philadelphia, in collaboration with Penn Medicine, also offers proton therapy to children at the Roberts Proton Therapy Center.