Ewing sarcoma

Overview

Ewing (YOO-ing) sarcoma is a rare type of cancer that occurs in bones or in the soft tissue around the bones. This cancer most often begins in the long bones of the pelvis, legs or arms, but it can occur in any bone. Less often, Ewing sarcoma starts in the soft tissues of the arms, legs, abdomen or other locations.

Major advancements in the treatment of Ewing sarcoma have significantly improved outcomes. After completion of treatment, people need lifelong monitoring for potential late effects of intense chemotherapy and radiation.

Ewing sarcoma is more common in teenagers and young adults, but it can occur at any age.

Symptoms

Signs and symptoms of Ewing sarcoma include:

  • Pain, swelling or tenderness near the affected area
  • Bone pain, which may worsen at night or with physical activity
  • Unexplained tiredness
  • Fever with no known cause
  • Unintended weight loss
  • Broken bone with no known cause

Causes

The cause of Ewing sarcoma is unknown. Though Ewing sarcoma arises from specific types of cells, it doesn't appear to be inherited.

Risk factors

No clear risk factors for Ewing sarcoma are known.

Complications

Ewing sarcoma can spread from where it started to other areas, making treatment and recovery more difficult. For example, the cancer can spread to other tissues, bone marrow, other bones or to the lungs. Ewing sarcoma can also return after treatment.

As with other types of serious cancer, aggressive chemotherapy and radiation for Ewing sarcoma can cause substantial side effects, both in the short and long term. The health care team takes steps to treat and manage these effects as best as possible. And it's important for you to learn what to watch for and contact your team with any concerns.

Prevention

Ewing sarcoma can't be prevented. This type of cancer is not inherited and there is no known link to any lifestyle or environmental issues.

Any signs or symptoms should be checked out as soon as possible.

Diagnosis

Typically, signs and symptoms lead parents to seek medical help for their child. Sometimes Ewing sarcoma is ultimately found when a child is brought in for treatment for what's thought to be a sprain or some other type of sports injury. Usually an X-ray shows a suspicious area. Then the doctor recommends one or more additional imaging tests to evaluate the area, such as:

  • Computerized tomography (CT)
  • Magnetic resonance imaging (MRI)
  • Positron emission tomography (PET)
  • Bone scan

Biopsy

The doctor may recommend a procedure to remove a sample of tissue (biopsy) from the tumor for lab testing. Testing can show whether the tissue is cancerous and, if so, what type of cancer. Testing also reveals the cancer's grade, which helps doctors understand how aggressive the cancer may be.

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 tumor. The needle is used to remove small pieces of tissue from the tumor.
  • Surgical biopsy. The doctor makes an incision through the skin and removes either the entire tumor (excisional biopsy) or a portion of the tumor (incisional biopsy).

Determining the type of biopsy needed and the specifics of how it should be performed requires careful planning by the medical team. Doctors need to perform the biopsy in a way that won't interfere with future surgery to remove the cancer. For this reason, ask your doctor for a referral to a team of experts with extensive experience in treating Ewing sarcoma before the biopsy.

After the diagnosis, the extent (stage) of the cancer needs to be determined — whether and how far it has spread. Localized Ewing sarcoma has not spread beyond its origin or nearby tissues. Metastatic Ewing sarcoma has spread to other areas of the body. The cancer stage guides treatment options.

Treatment

Over the years, major advances have been made in treating and curing cases of Ewing sarcoma. Treatment may include:

  • Chemotherapy. Chemotherapy, which uses drugs to kill cancer cells, is the first line of treatment. Doctors recommend chemotherapy before surgery to shrink Ewing sarcoma tumors and make them easier to remove.
  • Surgery. The goal of surgery is to remove the cancer cells, but surgeons also perform operations to maintain function and minimize disability. Surgery for Ewing sarcoma may involve removing a small portion of bone or removing an entire limb. The possibility of saving the limb depends on several factors, such as the tumor's size, location and its response to chemotherapy.
  • Radiation therapy. Radiation therapy uses high-energy beams, such as X-rays and protons, to kill cancer cells. It may be used after surgery to kill any cancer cells that remain. Radiation therapy can also be used instead of surgery if the sarcoma is located in a part of the body where surgery is not possible or would result in unacceptable functional outcomes (such as loss of bowel or bladder function). Radiation therapy is also used to relieve pain caused by Ewing sarcoma.
  • Clinical trials. Clinical trials are studies to investigate new ways of treating cancer. Many of the advances in treating pediatric cancers, including Ewing sarcoma, come from clinical trials of the Children's Oncology Group, which has more than 200 participating medical institutions from the United States and other countries.

Coping and support

A diagnosis of Ewing sarcoma can be frightening — especially for the family of a newly diagnosed child. With time you'll find ways to cope with the distress and uncertainty of cancer. Until then, you may find it helps to:

  • Learn enough about Ewing sarcoma to make decisions about care. Ask your doctor about Ewing sarcoma, including treatment options. As you learn more, you may become more confident in understanding and making decisions about treatment options. If your child has cancer, ask the health care team for guidance on sharing this information in a caring and age-appropriate way.
  • Keep friends and family close. Keeping your close relationships strong will help you deal with Ewing sarcoma. Friends and relatives can provide the practical support you'll need, such as helping take care of your house if your child is in hospital. And they can serve as emotional support when you feel overwhelmed.
  • Ask about mental health support. The concern and understanding of a counselor, medical social worker, psychologist or other mental health professional also may help you. If your child has cancer, ask your health care team for advice on providing emotional and social support and options for professional mental health support. You can also 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, you'll likely start by making an appointment with your primary care doctor — or the pediatrician if the concern is with your child. If your doctor suspects Ewing sarcoma, make sure you're 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 (orthopedic oncologists)
  • Other surgeons, depending on the location of the tumor and patient age (for example, chest surgeons or pediatric surgeons)
  • Doctors who specialize in treating cancer with chemotherapy or other systemic medications (medical oncologists or, for children, pediatric oncologists)
  • Doctors who use radiation to treat cancer (radiation oncologists)
  • Doctors who analyze tissue to diagnose the specific type of cancer (pathologists)
  • 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
  • Any medications being taken, including vitamins, herbs and over-the-counter medicines, and their dosages
  • Key personal information, including any major stresses or recent life changes

Also:

  • Bring previous scans or X-rays (both the images and the reports) and any other medical records related to this situation
  • Consider taking a relative or friend along to help you remember all the information provided during the appointment
  • Prepare a list of questions to ask the doctor to make the most of your time

Whether you're the patient or your child is the patient, 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 the ability to have children? If so, do you offer fertility preservation evaluations and services?
  • Are there any brochures or other printed material that I can have? What websites do you recommend?

What to expect from your doctor

Your doctor will likely ask several questions. Be ready to answer them to allow more time to cover other points you want to address. Whether you're the patient or your child is the patient, the doctor may ask:

  • What are the signs and symptoms that you're concerned about?
  • When did you first notice these symptoms?
  • Have the symptoms been continuous or occasional?
  • How severe are the symptoms?
  • What, if anything, seems to improve the symptoms?
  • What, if anything, appears to worsen the symptoms?

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