Ewing Sarcoma Treatment (PDQ®)–Patient Version

General Information About Ewing Sarcoma

KEY POINTS

Ewing sarcoma is a type of tumor that forms in bone or soft tissue.
Undifferentiated round cell sarcoma may also occur in the bone or soft tissue.
Signs and symptoms of Ewing sarcoma include swelling and pain near the tumor.
Tests that examine the bone and soft tissue are used to diagnose and stage Ewing sarcoma.
A biopsy is done to diagnose Ewing sarcoma.
Certain factors affect prognosis (chance of recovery).

Ewing sarcoma is a type of tumor that forms in bone or soft tissue.

Ewing sarcoma is a type of tumor that forms from a certain kind of cell in bone or soft tissue. Ewing sarcoma may be found in the bones of the legs, arms, feet, hands, chest, pelvis, spine, or skull. Ewing sarcoma also may be found in the soft tissue of the trunk, arms, legs, head, neck, retroperitoneum (area in the back of the abdomen behind the tissue that lines the abdominal wall and covers most of the organs in the abdomen), or other areas.

Ewing sarcoma is most common in adolescents and young adults (teens through mid-20s).

Ewing sarcoma has also been called peripheral primitive neuroectodermal tumor, Askin tumor (Ewing sarcoma of the chest wall), extraosseous Ewing sarcoma (Ewing sarcoma in tissue other than bone), and Ewing sarcoma family of tumors.

Undifferentiated round cell sarcoma may also occur in the bone or soft tissue.

Undifferentiated round cell sarcoma usually occurs in the bones or the muscles that are attached to bones and that help the body move. There are three types of undifferentiated round cell sarcoma that are treated like Ewing sarcoma:

Undifferentiated round cell sarcoma with BCOR-CCNB3 rearrangements. This type of bone tumor usually forms in the pelvis, arms, or legs. It may spread to other parts of the body. In this type of round cell sarcoma, the BCOR gene is joined to the CCNB3 gene. To diagnose round cell sarcoma, the tumor cells are checked for this gene change.
Undifferentiated round cell sarcoma with CIC-DUX4 rearrangements. This type of soft tissue tumor usually forms in the trunk, arms, or legs. It is most common in males and in young adults between 21 and 40 years of age. In this type of round cell sarcoma, the CIC gene is joined to the DUX4 gene. To diagnose round cell sarcoma, the tumor cells are checked for this gene change.
Undifferentiated round cell sarcoma with CIC-NUTM1 rearrangements. This type of soft tissue tumor usually forms in the central nervous system, but it can also form in the trunk. It is most common in younger patients.

Signs and symptoms of Ewing sarcoma include swelling and pain near the tumor.

These and other signs and symptoms may be caused by Ewing sarcoma or by other conditions. Check with your child’s doctor if your child has any of the following:

Pain and/or swelling, usually in the arms, legs, chest, back, or pelvis.
A lump (which may feel soft and warm) in the arms, legs, chest, or pelvis.
Fever for no known reason.
A bone that breaks for no known reason.

Tests that examine the bone and soft tissue are used to diagnose and stage Ewing sarcoma.

Procedures that make pictures of the bones and soft tissues and nearby areas help diagnose Ewing sarcoma and show how far the cancer has spread. The process used to find out if cancer cells have spread within and around the bones and soft tissues or to other parts of the body is called staging.

To plan treatment, it is important to know whether the cancer has spread to other parts of the body. Tests and procedures to detect, diagnose, and stage Ewing sarcoma are usually done at the same time.

The following tests and procedures may be used to diagnose or stage Ewing sarcoma:

Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the area where the tumor formed. This procedure is also called nuclear magnetic resonance imaging (NMRI).