Ewing’s Sarcoma

Ewing’s tumors are made up of small, undifferentiated round “blue” cells that are found in the body’s bones or soft tissues. The Ewing’s sarcoma family of tumors consist of Ewing’s sarcoma of the bone, extraosseous (outside the bone) Ewing’s sarcoma, and Askin tumors. Primitive neuroectodermal tumors (PNET) of the bone and PNET of the soft tissue. Ewing’s sarcoma is frequently found in one of the long bones (femur), the upper arm, the spine, the ribs, or the pelvic bones. 

Signs and symptoms

  • Swelling of bone or soft tissue over area of the tumor
  • Pain at the site of the tumor
  • If tumor located in pelvis, may have signs of bowel or bladder disturbance
  • If tumor located in spine, back pain, weakness in extremities or numbness can be present
  • If tumor located in chest wall, symptoms could be cough, shortness of breath or chest pain
  • A fracture (break) in bone in area of tumor (the break does not cause cancer rather than it is the result of the tumor)

Tests to help diagnose Ewing's Sarcoma

  • Physical exam and history: The health care provider will check general signs of health, assess for any lumps or anything else that seems unusual. They will also get history of past illnesses, health habits and any family history of illness or cancer.
  • CT scan: A computer assisted X-ray that shows detailed pictures inside the body, such as neck, chest, abdomen and pelvis. A dye may be injected into a vein or swallowed to help the organ and tissues show up more clearly.
  • MRI (magnetic resonance imaging): Is a test that uses magnet, radio waves and a computer to make a series of detailed pictures of areas inside the body.
  • X-rays: Plain film X-ray of the affected area and any other area of concern. 
  • PET scan (positron emission tomography): An X-ray that helps to show where the malignant tumor cells are in the body. A small amount of radioactive glucose (sugar) is injected into the vein. The scanner will then make pictures where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells. The PET scan has three steps:
    1. Injection of radioactive glucose
    2. A waiting period of 30-60 minutes
    3. Scanning by the PET machine
  • Bone marrow aspiration and biopsy: Sometimes done to help determine if the Ewing's sarcoma is in the bone marrow, the blood producing factory in the body. A special needle is inserted into one of the bones (typically the back of the hip bone) and a small amount of marrow (liquid part) is aspirated into a syringe. The biopsy includes taking a small piece of bone and sending it to lab for review. Since the procedure is uncomfortable most patients are given pain medicine or sedated during procedure.
  • Bone scan: Sometimes preformed to see if tumor has spread to any of the bones. A small amount of isotope (radioactive marker that makes any areas of the tumor in the bone light up on pictures) is injected into the vein. Approximately 2-3 hours later, pictures are taken.
  • Blood tests:
    • CBC (complete blood count) checks the number of red blood cells (oxygen carriers), platelets (cells that help the blood clot properly) and white blood cells (infection fighters)
    • Blood chemistry studies to check kidney and liver function.

All these tests are usually done at time of diagnosis to rule out diseases and are also done throughout treatment to monitor response and to monitor for possible side effects of treatment. 

  • Biopsy: There are different types of biopsies. The type of biopsy done will be based on the size of the tumor and where it is in the body
    • Incisional biopsy: Removal of part of a lump or a sample of tissue
    • Core biopsy: Removal of tissue using a wide needle

How is it treated?

Chemotherapy

Medications that are used to help kill cancer cells. Multiple types of chemotherapy medications are used in the treatment of Ewing’s sarcoma. Chemotherapy is given in a specific sequenced combination, given IV. Each chemotherapy treatment days is given in the hospital. The health care team will explain in detail the treatment plan and possible side effects of the chemotherapy.

Surgery

The timing of the surgery depends on the size and extent of tumor and the tumor’s response to chemotherapy, but it is often done after several rounds of chemotherapy have been given to help reduce tumor size. The type of surgery will depend on the location and the size of the tumor, whether the nerves and/or blood vessels are separate from the tumor, and the age of the child (how much more growth is expected). 

Radiation

Radiation therapy is a precise kind of X-ray treatment. Depending on where the tumor is located, radiation therapy may be used. It is given in specifically measured amounts by radiation therapy experts. If radiation therapy is necessary, your health care team along with the radiation therapy doctor will discuss exactly how the radiation is given and how long the treatments will last.