Ewing sarcoma

Ewing (Yoo-ing) sarcoma, also called Ewing's sarcoma, is a rare type of cancer that starts as a growth of cells in the bones or in the soft tissue around the bones. Ewing sarcoma happens most often in children, teenagers and young adults, but it can happen at any age.

Ewing sarcoma most often begins in bone, especially in the pelvis and long bones of the legs. But it can happen in any bone. Less often, it starts outside a bone in soft tissue. Ewing sarcoma is different from other bone cancers, such as osteosarcoma and chondrosarcoma.

Major advances in the treatment of Ewing sarcoma have improved the outlook for this cancer. If the cancer has not spread, treatment is more likely to be successful. But if the cancer has spread, it's more challenging to treat.

Ewing sarcoma 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, especially if the cancer has spread. These can include:

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

When to seek care

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

It is not clear what causes Ewing sarcoma.

Ewing sarcoma happens when cells develop changes in their DNA. A cell's DNA contains genes that give cells instructions for how to work, including when to grow and divide. Healthy cells follow signals that control growth and tell damaged or unneeded cells when to die.

In cancer cells, changes in DNA can disrupt these instructions. The cells may grow and divide without control and may survive when they should have died.

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's called metastatic cancer.

In Ewing sarcoma, the DNA changes most often affect a gene called EWSR1. In most cases in children, EWSR1 joins with FLI1, creating what's called a fusion of these genes. Testing tumor cells for these gene fusions can help confirm the diagnosis. These gene changes are not inherited.

The only known risk factors for Ewing sarcoma can't be changed:

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

Complications of Ewing sarcoma include cancer that spreads. The lungs, other bones and bone marrow are common places for Ewing sarcoma to spread.

Prevention

There is no known way to prevent Ewing sarcoma. Researchers have not found lifestyle or environmental factors that cause Ewing sarcoma. Gene changes, such as in the EWSR1 gene, found in Ewing sarcoma are not inherited.

Ewing sarcoma diagnosis usually begins with a physical exam and a review of symptoms and medical history. Imaging and biopsy are used to locate the tumor, find out whether it has spread and confirm the diagnosis.

Imaging tests

Imaging tests make pictures of the body. They can help show where Ewing sarcoma is located, how large it is and whether it has spread. Imaging may include:

• X-ray.
• MRI.
• CT scan.
• Bone scan.
• Positron emission tomography (PET) scan.

Removing a sample of cells for testing

A biopsy is needed to confirm the diagnosis of Ewing sarcoma. A biopsy is a procedure to remove a sample of tissue for testing in a lab. A radiologist or surgeon may place a needle through the skin and into the cancer to get a tissue sample or get a tissue sample during surgery. In the lab, a pathologist examines the sample for cancer and looks for features of Ewing sarcoma.

Your healthcare team uses the biopsy results, along with imaging and other tests, to make a treatment plan.

Genetic testing of cancer cells

A sample of the cancer cells is tested in the lab for certain gene changes called mutations. Ewing sarcoma cells mostly have changes in the EWSR1 gene. Most often, the EWSR1 gene joins with another gene called FLI1. This creates a gene fusion called EWSR1::FLI1. But other gene changes may occur with Ewing sarcoma. Testing the cancer cells for these gene changes can help confirm your diagnosis.

Because Ewing sarcoma is complex, care is best provided by a team of specialists with experience treating sarcoma. Ewing sarcoma treatment usually combines chemotherapy with treatment to control the tumor at its original site. The treatment may be surgery, radiation therapy or both.

Chemotherapy

Chemotherapy is a central part of Ewing sarcoma treatment. Chemotherapy treats cancer with strong medicines. It usually begins before surgery or radiation therapy. Chemotherapy continues after local treatment to treat cancer cells that may remain elsewhere in the body.

Chemotherapy may shrink the tumor before surgery or radiation therapy. That can make it easier to remove the tumor or target it with radiation therapy.

After surgery or radiation therapy, chemotherapy may be used to treat any cancer cells that may remain.

For advanced cancer that spreads to other areas of the body, chemotherapy remains an important part of treatment. Treatment plans also may include surgery or radiation therapy for some places where the cancer has spread.

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 tissues. Rarely, it might mean removing the affected arm or leg.

Surgery on an arm or leg might affect how you use that limb. Surgeons carefully plan the surgery to preserve function when possible.

Whether surgeons can remove all the cancer without removing an arm or leg depends on several factors. These include the tumor's size, 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 may be used before or after surgery. Or it might be used instead of surgery if surgery is not possible or could cause major loss of function. The choice to do radiation therapy depends on several factors. These factors include the tumor's size, where it is, its response to chemotherapy and the complexity of the surgery.

For Ewing sarcoma that has spread, radiation therapy may be used to treat selected sites or to help relieve symptoms such as pain.

Targeted therapy

Targeted therapies are designed to block specific molecules or pathways that cancer cells depend on. For Ewing sarcoma, these treatments are being studied mainly for disease that has come back or has not responded to chemotherapy. Some approaches have shown activity in early studies, but they are not commonly used as the first treatment option for Ewing sarcoma.

Long-term treatment side effects

Treatment for Ewing sarcoma can cause major short- and long-term side effects. Your healthcare team can help you manage the side effects that happen during treatment. The team also can discuss side effects to watch for in the years after treatment.

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.

Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.

Prognosis

Ewing sarcoma can be treated successfully when the cancer has not spread. The outlook, also called the prognosis, depends most strongly on whether the cancer has spread at diagnosis and where it has spread. Other factors include the tumor's size and location, the person's age at diagnosis, and how well the cancer responds to treatment. Talk with your healthcare team if you want to know more about the outlook for you or your child.

A diagnosis of Ewing sarcoma can feel overwhelming. With time you can 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 and possible side effects. 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 can help you deal with Ewing sarcoma. Ask friends and family to help with daily tasks, such as helping take care of your home. They can give emotional support when you need extra help.

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.

If there are 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 for a referral to an experienced specialist.

Ewing sarcoma typically needs to be diagnosed and 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 surgeon 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. For children, they might include 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.
• Child life specialists who can provide support for children and teens during cancer care.

What you can do

Before the appointment, make a list of:

• 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 side effects and risks of each treatment option?
• Could 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 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?

Stages describe how far Ewing sarcoma has spread. They help the healthcare team plan treatment. Healthcare professionals most often describe the stage of Ewing sarcoma as localized or metastatic:

• Localized Ewing sarcoma. The cancer has not spread to a distant part of the body. It's limited to the area where it started or in surrounding tissues such as muscles or tendons.
• Metastatic Ewing sarcoma. The cancer has spread from where it started to another part of the body. The most common sites of spread are the lungs, other bones or the bone marrow.

Though treatment discussions for Ewing sarcoma usually focus on whether the cancer is localized or metastatic, the American Joint Committee on Cancer (AJCC) uses tumor, nodes and metastasis (TNM) staging for bone sarcoma. The TNM staging takes into account the tumor grade, location, size, and whether the cancer has spread to lymph nodes or other parts of the body. AJCC TNM staging is used less often for Ewing sarcoma.

To find out whether Ewing sarcoma has spread, the healthcare team uses imaging such as MRI, a CT scan, a PET scan and sometimes a bone scan. A biopsy removes a sample of the tumor for testing. The biopsy can confirm the diagnosis of Ewing sarcoma.

Whether cancer has spread is one of the most important factors that affects the outlook, also called the prognosis.

Treatment usually includes chemotherapy and surgery. Sometimes radiation therapy is used before or after surgery, or instead of surgery.