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Overview

Ewing sarcoma is a type of cancer that begins as a growth of cells in the bones and the soft tissue around the bones. Ewing (Yoo-ing) sarcoma mostly happens in children and young adults, although it can happen at any age.

Ewing sarcoma most often begins in the leg bones and in the pelvis, but it can happen in any bone. Less often, it starts in the soft tissues of the chest, abdomen, arms or other locations.

Major advances in the treatment of Ewing sarcoma have improved the outlook for this cancer. Young people diagnosed with Ewing sarcoma are living longer. They sometimes face late effects from the strong treatments. Healthcare professionals often suggest long-term monitoring for side effects after treatment.

Symptoms

Ewing sarcoma signs and symptoms typically start in and around a bone. This cancer most often affects bones in the legs and the pelvis.

When symptoms happen in and around a bone, they might include:

• A lump in the arm, leg, chest or pelvis.
• Bone pain.
• Break in a bone, also called a fracture.
• Pain, swelling or tenderness near the affected area.

Sometimes Ewing sarcoma causes symptoms that affect the whole body. These can include:

• Fever.
• Losing weight without trying.
• Tiredness.

When to see a doctor

Make an appointment with a healthcare professional if you or your child has ongoing signs and symptoms that worry you.

Causes

It is not clear what causes Ewing sarcoma.

Ewing sarcoma happens when cells develop changes in their DNA. A cell's DNA holds the instructions that tell a cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time.

In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to make many more cells quickly. Cancer cells can keep living when healthy cells would die.

The cancer cells might form a mass called a tumor. The tumor can grow to invade and destroy healthy body tissue. In time, cancer cells can break away and spread to other parts of the body. When cancer spreads, it is called metastatic cancer.

In Ewing sarcoma, the DNA changes most often affect a gene called EWSR1. If your healthcare professional suspects that you or your child has Ewing sarcoma, the cancer cells may be tested to look for changes in this gene.

Risk factors

Risk factors for Ewing sarcoma include:

• Young age. Ewing sarcoma can happen at any age. But it is more likely to happen in children and young adults.
• European ancestry. Ewing sarcoma is more common in people of European ancestry. It's much less common in people of African and East Asian ancestry.

There's no way to prevent Ewing sarcoma.

Complications

Complications of Ewing sarcoma and its treatment include the following.

Cancer that spreads

Ewing sarcoma can spread from where it started to other areas. Ewing sarcoma most often spreads to the lungs and to other bones.

Long-term treatment side effects

The strong treatments needed to control Ewing sarcoma can cause major side effects, both in the short and long term. Your healthcare team can help you manage the side effects that happen during treatment. The team also can give you a list of side effects to watch for in the years after treatment.

Diagnosis

Ewing sarcoma diagnosis usually begins with a physical exam. Based on the findings of the exam, there might other tests and procedures.

Imaging tests

Imaging tests make pictures of the body. They can show the location and size of a Ewing sarcoma. Tests might include:

• X-ray.
• MRI.
• CT.
• Bone scan.
• Positron emission tomography scan, also called a PET scan.

Removing a sample of cells for testing

A biopsy is a procedure to remove a sample of tissue for testing in a lab. The tissue might be removed using a needle that is put through the skin and into the cancer. Sometimes surgery is needed to get the tissue sample. The sample is tested in a lab to see if it is cancer. Other special tests give more details about the cancer cells.

A biopsy is needed to confirm a Ewing sarcoma diagnosis. Your healthcare team uses this information to make a treatment plan.

Testing the cancer cells for DNA changes

A sample of the cancer cells will be tested in the lab to find which DNA changes are in the cells. Ewing sarcoma cells mostly have changes in the EWSR1 gene. Most often the EWSR1 gene joins with another gene called FLI1. This creates a new gene called EWS-FLI1.

Testing the cancer cells for these gene changes can help confirm your diagnosis.

Treatment

Ewing sarcoma treatment most often includes chemotherapy and surgery. Which treatment you have first will depend on your situation. Other treatment options might include radiation therapy and targeted therapy.

Chemotherapy

Chemotherapy treats cancer with strong medicines.

Chemotherapy is sometimes used as the first treatment for Ewing sarcoma. The medicines may shrink the cancer. That makes it easier to remove the cancer with surgery or target with radiation therapy.

After surgery or radiation therapy, chemotherapy treatments might be used to kill any cancer cells that might remain.

For advanced cancer that spreads to other areas of the body, chemotherapy might help relieve pain and slow the growth of the cancer.

Surgery

The goal of surgery is to remove all the cancer cells. Surgery for Ewing sarcoma might mean removing a small portion of bone and some surrounding tissue. Rarely, it might mean removing the affected arm or leg.

Surgery on an arm or leg might affect the way you can use that limb. Surgeons carefully plan the surgery to minimize this risk, when possible.

Whether surgeons can remove all the cancer without removing the arm or leg depends on several factors. These include the size of the cancer, where it is and whether chemotherapy helps shrink it.

Radiation therapy

Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body.

Radiation therapy might be suggested after surgery to kill cancer cells that remain. Radiation therapy might be used instead of surgery if an operation is not possible or if it is likely to hurt nearby organs. For example, if the surgery might cause loss of bowel or bladder control, radiation might be used instead.

For advanced Ewing sarcoma, radiation therapy can slow the growth of the cancer and help relieve pain.

Targeted therapy

Targeted therapy for cancer is a treatment that uses medicines that attack specific ways that cancer cells can grow. By blocking these specific things in the cells, targeted treatments can cause cancer cells to die. For Ewing sarcoma, researchers are looking at using targeted therapy when the cancer comes back or does not respond to other treatments.

Clinical trials

Clinical trials are studies of new treatments. These studies provide a chance to try the latest treatments. The risk of side effects might not be known. Ask your healthcare team if you or your child might be able to join a clinical trial.

Coping and support

A diagnosis of Ewing sarcoma can feel overwhelming. With time you will find ways to cope with the distress and uncertainty of cancer. Until then, you may find these suggestions helpful.

Learn about Ewing sarcoma to make decisions about care

Ask your or your child's healthcare professional about Ewing sarcoma, including treatment options. As you learn more, you may feel better about making choices about treatment options. If your child has Ewing sarcoma, ask the healthcare team to guide you in talking to your child about the cancer in a caring way that your child can understand.

Keep friends and family close

Keeping your close relationships strong will help you deal with Ewing sarcoma. Friends and family can help with daily tasks, such as helping take care of your home if your child is in the hospital. They can serve as emotional support when you feel like you are dealing with more than you can handle.

Ask about mental health support

Talking to a counselor, medical social worker, psychologist or other mental health professional also may help you or your child. Ask your healthcare team for options for professional mental health support for you and your child. You also can check online for a cancer organization, such as the American Cancer Society, that lists support services.

Preparing for an appointment

If there are signs and symptoms that worry you, start by making an appointment with a healthcare professional for you or your child. If the health professional suspects Ewing sarcoma, ask to be referred to an experienced specialist.

Ewing sarcoma typically needs to be treated by a team of specialists, which may include:

• Orthopedic surgeons who specialize in operating on cancers that affect the bones, called orthopedic oncologists.
• Other surgeons, such as chest surgeons or pediatric surgeons. The type of surgeons depends on the cancer's location and the age of the person with Ewing sarcoma.
• Doctors who specialize in treating cancer with chemotherapy or other medicines. They might include medical oncologists or, for children, pediatric oncologists.
• Doctors who study tissue to diagnose the type of cancer, called pathologists.
• Doctors who use radiation to treat cancer, called radiation oncologists.
• Rehabilitation specialists who can help in recovery after surgery.

What you can do

Before the appointment, make a list of:

• Signs and symptoms, including any that seem unrelated to the reason for the appointment, and when they began.
• Any medicines you or your child takes, including vitamins and herbal supplements, and their doses.
• Key personal information, including any major stresses or recent life changes.

Also:

• Bring scans or X-rays, both the images and the reports, and any other medical records that are linked to this condition.
• Make a list of questions to ask the health professional to make sure you get the information you need.
• Take a relative or friend to the appointment, if you can, to help you remember the information you get.

For you or your child, your questions might include, for example:

• What type of cancer is this?
• Has the cancer spread?
• Are more tests needed?
• What are the treatment options?
• What are the chances that treatment will cure this cancer?
• What are the side effects and risks of each treatment option?
• Will treatment affect being able to have children? If so, do you offer ways to be able to preserve that ability?
• Are there brochures or other printed material I can have? What websites do you suggest?

What to expect from your doctor

Your healthcare professional will likely ask you questions, such as:

• What are the signs and symptoms that worry you?
• When did you notice these symptoms?
• Do you always have the symptoms, or do they come and go?
• How severe are the symptoms?
• What, if anything, seems to improve the symptoms?
• What, if anything, appears to worsen the symptoms?