What is Ewing sarcoma?
Ewing sarcoma is a cancer that occurs primarily in the bone or soft tissue. Ewing sarcoma can occur in any bone, but is most often found in the extremities and can involve muscle and the soft tissues around the tumor site. Ewing sarcoma cells can also spread (metastasize) to other areas of the body including the bone marrow, lungs, kidneys, heart, adrenal gland, and other soft tissues. Ewing sarcoma accounts for about 1.8 percent of childhood cancers. About 150 children and adolescents are diagnosed with Ewing sarcoma each year in the US. It is the second most common malignant bone tumor in children and adolescents. Ewing sarcoma most often occurs in children between the ages of 5 and 20. The number of males affected is slightly higher than the number of females.
What causes Ewing sarcoma?
The majority of Ewing sarcomas result from a chromosome rearrangement between chromosomes #11 and #22. This rearrangement changes the position and function of genes, causing a fusion of genes referred to as a fusion transcript. Over 90 percent of individuals have an abnormal fusion transcript, involving two genes known as EWS and FLI1. This important discovery has led to improvements in diagnosing Ewing sarcoma. Similar to osteogenic sarcoma, trauma or injury is sometimes involved with the site at the time of diagnosis. However, this trauma is thought to bring the condition to attention rather than to have any causal relationship. Some physicians classify Ewing sarcoma as a primitive neuroectodermal tumor (PNET). This means the tumor may have started in fetal, or embryonic, tissue that has developed into nerve tissue.
What are the symptoms of Ewing sarcoma?
The following are the most common symptoms of Ewing sarcoma. However, each child may experience symptoms differently. Symptoms may include, but are not limited, to the following:
- Pain around the site of the tumor
- Swelling and/or redness around the site of the tumor
- Weight loss, decreased appetite
- Paralysis and/or incontinence if the tumor is in the spinal region
- Numbness, tingling, paralysis, and other symptoms related to nerve compression from the tumor
The symptoms of Ewing sarcoma may resemble other conditions or medical problems. Always consult your child’s physician for a diagnosis.
How is Ewing sarcoma diagnosed?
In addition to a complete medical history and physical examination of your child, diagnostic procedures for Ewing sarcoma may include:
- X-rays use invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film. This test is used to measure and evaluate the curve.
- Bone scans are a nuclear imaging method that evaluates any degenerative and/or arthritic changes in the joints. Bone scans detect bone diseases and tumors, determine the cause of bone pain or inflammation and rule out any infection or fractures.
- Magnetic resonance imaging (MRI) – is a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. This test is done to rule out any associated abnormalities of the spinal cord and nerves.
- Computer-assisted tomography scan (CT or CAT scan) is a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
- Blood tests, including blood chemistries.
- Biopsy of the tumor, which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
- Bone marrow aspiration and/or biopsy. This procedure involves taking a small amount of bone marrow fluid (aspiration) and/or solid bone marrow tissue (called a core biopsy), usually from the hip bones, to be examined for the number, size, and maturity of blood cells and/or abnormal cells.
Ewing sarcoma is difficult to distinguish from other similar tumors. Diagnosis is often made by excluding all other common solid tumors, and by the use of genetic studies.
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