Key Points for This Section

Ewing family of tumors is a group of cancers of the bone and of soft tissue. Possible signs of Ewing family of tumors include swelling and pain near the tumor. Tests that examine the bone and soft tissue are used to diagnose and stage Ewing family of tumors. A biopsy is done to diagnose Ewing family of tumors. Certain factors affect prognosis (chance of recovery) and treatment options.

Ewing family of tumors is a group of cancers of the bone and of soft tissue.

Ewing family of tumors is a group of tumors that form from a certain kind of cell in bone or soft tissue. This family of tumors includes the following:

• Ewing tumor of the bone. This type of tumor is found in the bones of the legs, arms, chest, trunk, back, or head. There are three types of Ewing tumor of bone:
  • Classic Ewing sarcoma.
  • Primitive neuroectodermal tumor (PNET).
  • Askin tumor (PNET of the chest wall).
• Extraosseous Ewing sarcoma (tumor growing in tissue other than bone). This type of soft tissue tumor is found in the trunk, arms, legs, head, and neck.

Ewing tumors usually occur in teenagers and are more common in boys and Caucasians.

Possible signs of Ewing family of tumors include swelling and pain near the tumor.

These and other symptoms may be caused by Ewing family of tumors. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

• Pain and/or swelling, most commonly in the arms, legs, chest, back, or pelvis (area between the hips).
• A lump (which may feel 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 family of tumors.

The following tests and procedures may be used to diagnose or stage Ewing family of tumors:

• Physical exam and 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.
• Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
  • The number of red blood cells, white blood cells, and platelets.
  • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
  • The portion of the blood sample made up of red blood cells.
• Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances, such as lactate dehydrogenase (LDH), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
• Sedimentation rate: A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube.
• X-ray: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
• 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. This procedure is also called nuclear magnetic resonance imaging (NMRI).
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
• Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone. Samples are removed from both hipbones. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
• Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.
• PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.

A biopsy is done to diagnose Ewing family of tumors.

Cells and tissues are removed during a biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. The specialists (pathologist, radiation oncologist, and surgeon) who will treat the patient usually work together to plan the biopsy. This is done so that the biopsy incision doesn't affect later treatment with surgery to remove the tumor and radiation therapy. It is helpful if the biopsy is done at the same center where treatment will be given.

The following tests may be done on the tissue that is removed:

• Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
• Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
• Reverse-transcription polymerase chain reaction test (RT-PCR): A laboratory test in which cells in a sample of tissue are studied using chemicals to look for certain changes in the genes.
• Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) depends on certain factors before and after treatment.

Before treatment, prognosis depends on:

• Where in the body the tumor started.
• How large the tumor is at diagnosis.
• Whether the tumor has spread to distant parts of the body.
• Whether the tumor has certain genetic changes.
• The patient's age, gender, and general health.
• Whether the tumor has just been diagnosed or has recurred (come back).

After treatment, prognosis is affected by:

• Whether the tumor was completely removed by surgery.
• Whether the cancer came back more than two years after the initial treatment.

Treatment options depend on the following:

• Where the tumor is found in the body and how large the tumor is.
• The patient's age and general health.
• The effect the treatment will have on the patient's appearance and important body functions.
• Whether the cancer has just been diagnosed or has recurred (come back).

Decisions about surgery may depend on how well the initial treatment with chemotherapy or radiation therapy works.

Glossary Terms

A type of protein made by plasma cells (a type of white blood cell) in response to an antigen (foreign substance). Each antibody can bind to only one specific antigen. The purpose of this binding is to help destroy the antigen. Antibodies can work in several ways, depending on the nature of the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen.

A substance that causes the immune system to make a specific immune response.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

A procedure in which a sample of blood is examined to measure the amounts of certain substances made in the body. An abnormal amount of a substance can be a sign of disease in the organ or tissue that produces it.

The soft, sponge-like tissue in the center of most bones. It produces white blood cells, red blood cells, and platelets.

The removal of a small sample of bone marrow (usually from the hip) through a needle for examination under a microscope.

The removal of a sample of tissue from the bone marrow with a needle for examination under a microscope.

A technique to create images of bones on a computer screen or on film. A small amount of radioactive material is injected into a blood vessel and travels through the bloodstream; it collects in the bones and is detected by a scanner.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Treatment with drugs that kill cancer cells.

The muscles, bones, and joints that make up the area of the body between the neck and the abdomen.

Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.

CBC. A test to check the number of red blood cells, white blood cells, and platelets in a sample of blood. Also called blood cell count and CBC.

A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.

Computed tomography scan. A series of detailed pictures of areas inside the body taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called computed tomography scan, computerized tomography, computerized axial tomography scan, and CAT scan.

The study of chromosomes and chromosomal abnormalities.

The process of identifying a disease, such as cancer, from its signs and symptoms.

A microscope (device used to magnify small objects) that uses electrons (instead of light) to produce an enlarged image. An electron microscope shows tiny details better than any other type of microscope.

A group of cancers that includes Ewing tumor of bone (ETB or Ewing sarcoma of bone), extraosseous Ewing (EOE) tumors, primitive neuroectodermal tumors (PNET or peripheral neuroepithelioma), and Askin tumors (PNET of the chest wall). These tumors all come from the same type of stem cell. Also called EFTs.

A type of cancer that forms in bone or soft tissue. Also called peripheral primitive neuroectodermal tumor and pPNET.

Located outside of the bone.

An increase in body temperature above normal (98.6 degrees F), usually caused by disease.

The functional and physical unit of heredity passed from parent to offspring. Genes are pieces of DNA, and most genes contain the information for making a specific protein.

Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.

A type of sugar; the chief source of energy for living organisms.

The substance inside red blood cells that binds to oxygen in the lungs and carries it to the tissues.

A cut made in the body to perform surgery.

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

A medical procedure that involves testing a sample of blood, urine, or other substance from the body. Tests can help determine a diagnosis, plan treatment, check to see if treatment is working, or monitor the disease over time.

One of a group of enzymes found in the blood and other body tissues and involved in energy production in cells. An increased amount of lactate dehydrogenase in the blood may be a sign of tissue damage and some types of cancer or other diseases. Also called lactic acid dehydrogenase and LDH.

Cancerous. Malignant tumors can invade and destroy nearby tissue and spread to other parts of the body.

A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, nuclear magnetic resonance imaging, and NMRI.

A part of the body that performs a specific function. For example, the heart is an organ.

A colorless, odorless gas. It is needed for animal and plant life. Oxygen that is breathed in enters the blood from the lungs and travels to the tissues.

A doctor who identifies diseases by studying cells and tissues under a microscope.

The lower part of the abdomen, located between the hip bones.

Positron emission tomography scan. A procedure in which a small amount of radioactive glucose (sugar) is injected into a vein, and a scanner is used to make detailed, computerized pictures of areas inside the body where the glucose is used. Because cancer cells often use more glucose than normal cells, the pictures can be used to find cancer cells in the body. Also called positron emission tomography scan.

An exam of the body to check for general signs of disease.

A type of blood cell that helps prevent bleeding by causing blood clots to form. Also called a thrombocyte.

PCR. A laboratory method used to make many copies of a specific DNA sequence. Also called PCR.

One of a group of cancers that develop from the same type of early cells, and share certain biochemical and genetic features. Some primitive neuroectodermal tumors develop in the brain and central nervous system (CNS-PNET), and others develop in sites outside of the brain such as the limbs, pelvis, and chest wall (peripheral PNET). Also called PNET.

The likely outcome or course of a disease; the chance of recovery or recurrence.

A molecule made up of amino acids that are needed for the body to function properly. Proteins are the basis of body structures such as skin and hair and of substances such as enzymes, cytokines, and antibodies.

A doctor who specializes in using radiation to treat cancer.

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called radiotherapy and irradiation.

Giving off radiation.

An unstable form of a chemical element that releases radiation as it breaks down and becomes more stable. Radioisotopes may occur in nature or be made in a laboratory. In medicine, they are used in imaging tests and in treatment. Also called radionuclide.

To come back or to return.

RBC. A cell that carries oxygen to all parts of the body. Also called RBC and erythrocyte.

In medicine, an instrument that takes pictures of the inside of the body.

The distance red blood cells travel in one hour in a sample of blood as they settle to the bottom of a test tube. The sedimentation rate is increased in inflammation, infection, cancer, rheumatic diseases, and diseases of the blood and bone marrow. Also called erythrocyte sedimentation rate and ESR.

Refers to muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.

A doctor who removes or repairs a part of the body by operating on the patient.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A group or layer of cells that work together to perform a specific function.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancerous), or malignant (cancerous). Also called neoplasm.

A blood vessel that carries blood to the heart from tissues and organs in the body.

Refers to a blood cell that does not contain hemoglobin. White blood cells include lymphocytes, neutrophils, eosinophils, macrophages, and mast cells. These cells are made by bone marrow and help the body fight infections and other diseases. Also called WBC.

A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.