The Ewing family of tumors is a group of cancers that start in the bones or nearby soft tissues that share some common features. These tumors can develop at any age, but they are most common in the early teen years.

The main types of Ewing tumors are:

  • Ewing sarcoma of bone: Ewing sarcoma that starts in a bone is the most common tumor in this family. This type of tumor was first described by Dr. James Ewing in 1921, who found it was different from the more common bone tumor, osteosarcoma. Seen under a microscope, its cells looked different from osteosarcoma cells. It was also more likely to respond to radiation therapy.
  • Extraosseous Ewing tumor (EOE): Extraosseous (not arising from bones) Ewing tumors start in soft tissues around bones, but they look and act very much like Ewing sarcomas in bones. They are also known as extraskeletal Ewing sarcomas.
  • Peripheral primitive neuroectodermal tumor (PPNET): This rare childhood cancer also starts in bone or soft tissue and shares many features with Ewing sarcoma of bone and EOE. Peripheral PNETs that start in the chest wall are known as Askin tumors. (Peripheral PNETs are similar to, but not quite the same as, PNETs of the brain and spinal cord.

Most Ewing tumors occur in the bones. The most common sites are:

  • The pelvis (hip bones)
  • The chest wall (such as the ribs or shoulder blades)
  • The legs, mainly in the middle of the long bones

Though usually, they arise in one part of the body, it is uncommon to have multiple sites involved at the same time. They are either spread from on the original sites or can arise from many sites at the same time.

Most Ewing tumors occur in children and teens, but they can also occur in adults.

Diagnosis is doen by a proper biopsy and review by an experienced onco-pathologist. It may be necessary to perform special tests of immunohistochemistry (IHC) and some molecular tests. Staging is done by CT scans, PET scans and MRI where necessary.

Once a Ewing tumor has been found and staged, the cancer care team will talk with you about treatment options. It’s important to be sure you understand your child’s options as well as their possible side effects to help make the decision that’s the best fit for your child. If there is anything you don’t understand, ask to have it explained. You can find some good questions to ask in “ What should you ask the doctor about Ewing tumors?”

The main goals of treatment of Ewing tumors are:

  • To try to cure the patient
  • To keep as much function in affected parts of the body as possible
  • To limit the long-term complications of treatment as much as possible

For children and teens, a team approach is recommended that includes the child’s pediatrician as well as children’s cancer specialists. Treatment for children and teens is best done at a children’s cancer center. For adults with Ewing tumors, the treatment team typically includes the patient’s primary care doctor, as well as specialists at a major cancer center. Doctors on the treatment team might include:

  • An orthopedic surgeon (a surgeon who specializes in muscles and bones) who is experienced in treating bone tumors
  • A medical or pediatric oncologist (a doctor who treats cancer with chemotherapy and other drugs)
  • A radiation oncologist (a doctor who treats cancer with radiation therapy)
  • A pathologist (a doctor specializing in lab tests to diagnose and classify diseases)
  • A physiotherapist (a doctor who directs a person’s rehabilitation and physical therapy)

For adults and children, the team will also include other doctors, physician assistants (PAs), nurse practitioners (NPs), nurses, psychologists, social workers, physical therapists and other rehabilitation specialists, and other health professionals. Going through cancer treatment often means meeting lots of specialists and learning about parts of the medical system you probably haven’t been exposed to before.

Before treatment, the doctors and other members of the team will help you, as a parent, understand the tests that will need to be done. The team’s social worker will also counsel you about the problems you and your child might have during and after treatments such as surgery, and might be able to help you find housing and financial aid if needed.

The types of treatment that can be used in Ewing tumors include:

  • Chemotherapy
  • Surgery
  • Radiation therapy
  • High-dose chemotherapy followed by a stem cell transplant

Chemotherapy is almost always the first treatment. Localized therapy (surgery and/or radiation therapy) is next, often followed by more chemotherapy. A stem cell transplant might be an option for some patients with Ewing tumors that are unlikely to be cured with other treatments.