A Brief Outline of Ewing’s Sarcoma
Ewing’s sarcoma is a very rare type of cancerous tumour that grows in your bones or the soft tissue around your bones, such as cartilage or the nerves. It usually affects people from the ages of 10 to 20 and has a high rate of being cured.
The majority of Ewing's 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% 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's 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 doctors classify Ewing's sarcoma as a primitive neuroectodermal tumour (PNET). This means the tumour may have started in fetal, or embryonic, tissue that has developed into nerve tissue.
Risk Factors for Ewing's Sarcoma
There is no way to know for sure if you're going to get Ewing's sarcoma. And there is no known way to prevent it.
Certain factors can make you more likely to get cancer than another person. There have not been any lifestyle (such as smoking) or inherited risk factors that have been directly linked to Ewing's sarcoma. However, there are a few general risk factors for getting Ewing's sarcoma.
General risk factors for Ewing's sarcoma
Ewing's sarcoma is most common in people who are between 10 and 20 years old.
Ewing's sarcoma is more common in white people than in black people or other ethnic groups.
Ewing's sarcoma affects males slightly more frequently than females.
You may have all of these general risk factors and not get Ewing's sarcoma. Or you may have none of these factors and get this type of cancer.
- Stiffness, pain, swelling, or tenderness in the bone or in the tissue surrounding the bone. About 85% of children and young adults with Ewing sarcoma have pain that can come and go and be less severe at night.
- A lump near the surface of the skin that may feel warm and soft to the touch.
- A fever that does not go away.
- A broken bone that happens without an injury. A tumour growing in the bone can cause the bone to become weak or fracture.
This may include:
- Imaging tests
- Removing a sample of cells for testing (biopsy)
- Testing the cancer cells for gene mutations
- Computerized tomography (CT)
- Magnetic resonance imaging (MRI)
- Positron emission tomography (PET)
- Bone scan
Removing a sample of cells for testing (biopsy):
Types of biopsy procedures used to diagnose Ewing sarcoma include:
Needle biopsy. The doctor inserts a thin needle through the skin and guides it into the tumour. The needle is used to remove small pieces of tissue from the tumour.
Surgical biopsy. The doctor makes an incision through the skin and removes either the entire tumour (excisional biopsy) or a portion of the tumour (incisional biopsy).
Testing the cancer cells for gene mutations
A sample of your cancer cells will be tested in the lab to determine which DNA changes are present in the cells. Ewing sarcoma cells usually have changes in the EWSR1 gene. Most often the EWSR1 gene becomes fused with another gene called FLI1, creating a new gene called EWS-FLI1. Testing the cancer cells for these gene changes can help confirm your diagnosis and give your doctor clues about the aggressiveness of your disease.
Types of Ewing’s Sarcoma
The kind of Ewing’s sarcoma you might have can be seen where the tumour forms. The pelvis is where it most often starts, followed by the femur (or thigh bone). Regardless of where it starts, it can spread to other bones, bone marrow, and even vital organs such as the lungs, heart, and kidneys. Some types are:
Bone tumour: 87% of Ewing’s sarcoma happens in the bone, often appearing in the thigh bones, pelvis, ribs, or shoulder blades. But the tumour can appear in any bone.
Soft tissue (extra-osseous) tumour: This type of tumour affects the soft tissues around your bones, such as cartilage or muscle. It’s rarely found in the soft tissue of the arms, legs, head, neck, chest, and abdomen.
Peripheral primitive neuro ectodermal tumour (pPNET): This type of tumour is found in the nerves and can be detected in many parts of the body.
A skin tumour: This is a type of pPNET tumour that’s found in the chest.
What kind of treatment you get will depend on several things. They include:
The size of your tumour
Where it has spread
Your overall health
Treatment options include:
Chemotherapy: This is usually the first step. With this option, medicines are used to kill cancer cells and stop them from growing. They can be injected into your bloodstream. Your doctor may use more than one type of chemotherapy at a time or combine this with surgery and radiation.
Surgery: Your doctor will try to remove the tumour to stop its spread. In some cases, she may have to amputate an arm or leg if the tumour has spread a lot.
In this therapy, a technician will use X-rays and other types of radiation to kill the cancer cells. This can be done using machines outside the body to deliver the dose, or through needles and tubes sent right to the tumour.
If the treatment works, you will still need to follow up with your doctor for many years. Ewing’s sarcoma can return even a decade after the diagnosis.
Children with Ewing’s sarcoma that hasn’t spread have cure rates as high as 80%. It’s much lower in cases where the tumour has spread. It’s important to see a doctor right away if you see symptoms in your child.
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