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Ewing Sarcoma

Ewing’s sarcoma is an aggressive, malignant (cancerous) tumor that typically affects older children and teenagers. It usually starts in the leg and arm bones but may spread to other parts of the body, including bone marrow, lungs, and other soft tissues.

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What Is Ewing Sarcoma?

Doctors define Ewing sarcoma as a malignant tumor that begins in a bone. The tumor most often occurs in the large bones of the body.

Common tumor sites include the:

  • Thigh
  • Shin
  • Upper arm
  • Ribs
  • Shoulder blades
  • Pelvis

Less frequently, Ewing sarcoma begins in the muscles, cartilage, or nerves near the bone.

Ewing sarcoma most often occurs in older children and teens. Very rarely, it can form in younger children or older adults. 

It's a serious type of cancer that often spreads to other parts of the body.

How common is Ewing sarcoma?

Ewing’s sarcoma is rare, making up about 1% of childhood cancers according to the American Cancer Society. Doctors diagnose about 200 children and teens with Ewing's sarcoma in the U.S. each year.

What are the types of Ewing sarcoma?

There are four types of Ewing sarcoma:

  • Ewing's sarcoma of bone, which is the most common Ewing's tumor.
  • Extraosseous Ewing's tumor, which starts in the soft tissue around the bone.
  • Peripheral primitive neuroectodermal tumor (PPNT), which can occur in both bone and soft tissue.
  • Askin’s tumor, which is a PPNT in the bones of the chest.

What causes Ewing sarcoma? 

Doctors don’t know what causes Ewing sarcoma to form.

They do know that the tumor begins when a cell’s chromosomes change. In Ewing's sarcoma cancer cells, the genetic material (DNA) in two specific chromosomes (11 and 22) gets mismatched. But doctors don’t know why this leads to a tumor forming.

What are Ewing sarcoma risk factors and complications?

Ewing sarcoma can occur at any age but is most common in school-age children and teenagers.

Ewing sarcoma risk factors 

There are no known risk factors for Ewing sarcoma. Doctors haven’t found any clear links between Ewing's tumors and lifestyle, diet, genetics, chemicals, or other environmental factors.

Ewing's tumors are slightly more common in males than in females.

Complications of Ewing sarcoma

The main complication of Ewing sarcoma is that it often metastasizes or spreads to other parts of the body — most commonly to the lungs, other bones, and bone marrow. If the cancer metastasizes, it can eventually lead to death.

How can I prevent Ewing sarcoma?

There's no known way to prevent getting Ewing sarcoma. If your child or teen develops an Ewing's tumor, know that it didn’t happen because of something you did or didn’t do.

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What Are the Signs and Symptoms of Ewing Sarcoma?

The symptoms of Ewing sarcoma aren’t always apparent right away. It’s easy to confuse them with other health issues, or with the typical minor injuries of childhood.

Signs of Ewing sarcoma include:

  • A bone fracture with no apparent cause. A bone weakened by a tumor may break after a seemingly harmless bump or bruise.
  • A lump or swelling near the surface of the skin. It may feel warm and soft to the touch.
  • Fever.
  • Pain near the tumor. The pain might get worse over time, especially at night or during activity.
  • Shortness of breath if the tumors have spread to the lungs.   
  • Stiffness in the area near the tumor.
  • Tenderness on the bone or surrounding tissue.
  • Weight loss.

When should my child see a doctor about Ewing sarcoma symptoms?

Your child should see a doctor for any of the above signs, especially if symptoms last longer than a few days.

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How Do You Diagnose Ewing Sarcoma?

If your child's doctor suspects Ewing sarcoma, they'll take a complete medical history. They'll ask about any symptoms your child may have, and how long they’ve been going on.

The doctor will also do a physical exam, focusing on the swollen or painful area.

Tests to diagnose Ewing sarcoma

If the doctor thinks there might be a bone tumor, they'll order imaging and lab tests. These tests may include:

If the tumor is Ewing sarcoma, your doctor will order more tests to see if the cancer has spread. Doctors call this process "staging." Once they find out the stage of the tumor, they can decide on the best treatment strategy.

Staging tests may include:

  • Blood tests.
  • Bone marrow biopsy.
  • CT scan of lungs.

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How Do You Treat Ewing Sarcoma?

Your child's treatment plan for Ewing’s sarcoma will depend on the location and stage of their tumor. A treatment team will decide on the best course of action for your child.

The treatments for Ewing’s sarcoma are chemotherapy, radiation, and surgery. They're often used in combination with each other.

The goals of treatment include:

  • Curing the cancer.
  • Keeping as much function in the affected body parts as possible.
  • Limiting long-term complications of treatment.

Your child’s treatment team may include:

  • Orthopaedic surgical oncologists.
  • Pathologists.
  • Pediatric medical oncologists.
  • Radiation oncologists.
  • Radiologists.

Medicine to treat Ewing sarcoma 

Chemotherapy is typically the first treatment doctors use on Ewing sarcoma. Doctors may use chemotherapy to kill the tumor and help stop the spread of the cancer. Chemotherapy can also help shrink a tumor before surgery, to make the operation easier.

What happens during chemotherapy treatment?

Doctors administer chemotherapy for Ewing sarcoma in a series of cycles. There's a rest period between cycles of chemotherapy to allow your child’s body to recover from the treatment.

Chemotherapy for a Ewing's tumor is a lengthy process that may take up to a year to complete. Doctors will insert a thin tube into the chest to deliver the treatment. The tube will stay in place until the end of treatment.

Chemotherapy uses combinations of drugs to kill cancer cells. But because these drugs affect the whole body, chemotherapy can also damage healthy cells. Side effects include hair loss, nausea, fevers, and mouth sores.

Radiation therapy to treat Ewing sarcoma 

Radiation therapy uses high-energy beams from a machine to shrink or kill cancer cells. This treatment can lower the chances of the primary tumor coming back.

Doctors may use radiation therapy with or instead of surgery. They may combine radiation with chemotherapy before, during, or after surgery for Ewing’s sarcoma.  

What happens during radiation therapy?

Doctors administer radiation therapy through beams outside the body. Generally, people receive treatments five days a week for several weeks. The treatment isn't painful and is like getting an x-ray.

The treatments only last a few minutes, but you must remain very still while it’s happening. Younger children may need medicine to put them to sleep so they don’t move around during treatment.

Possible side effects of radiation include:

  • Diarrhea.
  • Dry mouth or mouth sores.
  • Hair loss.
  • Long-term damage to lungs, heart, bowels, and other organs.
  • Lowered blood cell counts.
  • Nausea.
  • Skin reactions like sunburn.
  • Slowed bone growth.
  • Urinary problems.

Surgery for Ewing sarcoma

Doctors use surgery, if possible, to remove Ewing's tumors. They also remove some healthy surrounding tissue to keep the cancer from growing. Even a small amount of cancer left behind can grow and begin spreading again.

What happens during surgery for Ewing sarcoma?

Doctors use different types of surgery for Ewing's tumors. Tumors in the base of the skull, chest wall, spine, and pelvis are harder to remove. 

For most tumors in the arms and legs, doctors use limb-sparing surgery to remove all or part of the affected bone. They replace it with a piece of bone from another person or another part of your child's body, or a prosthetic device. The goal is to restore the limb so it can function normally.

These are long and complex surgeries. Surgeons must be able to remove the tumor while saving the nearby blood vessels, nerves, and tendons. Sometimes, amputating a limb is the only option. 

If surgery isn’t possible because the tumor extends into nerves or blood vessels, doctors may use radiation therapy instead. 

Side effects of Ewing tumor surgery include:

  • Blood clots.
  • Breaking or loosening of bone grafts or prostheses.
  • Excess bleeding.
  • Infections.
  • Pain.
  • Reactions to anesthesia.

Physical therapy and rehabilitation are necessary after Ewing's tumor surgery.

How effective is treatment?

The outcomes for people with Ewing sarcoma have gotten better over the years. Modern medicine and surgical techniques have greatly improved recently. Two-thirds of people without any spread of cancer will survive at least five years after diagnosis.  

The chance of a complete recovery is better if:

  • Doctors were able to completely remove the tumor.
  • The cancer didn’t spread.
  • The tumor was in the arms or legs instead of the pelvis.
  • Your child responded well to chemotherapy.

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