Orthopedic Surgery

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Ewing Sarcoma

Ewing sarcoma is a very rare form of bone cancer that strikes children, adolescents and young adults. Actually, it is a group of four different types of cancer, known collectively as the Ewing's family of tumors.

It is a malignant form of cancer — that is, it has a tendency to get worse and can spread quickly to other organs. Approximately 250 cases are diagnosed annually in the United States.

Although it can occur at a younger age, most Ewing sarcoma cases are found in teenagers. Caucasian children, particularly boys, are far more likely to have Ewing sarcoma than any other racial or ethnic group.

Causes of Ewing sarcoma

Ewing sarcomas are caused by chromosome changes that occur after birth. In a process known as translocation, chromosomes 11 and 22 “swap” small pieces of each other. This result is an abnormal gene that can be detected with DNA testing.

It is not known why translocation occurs. In a family, one sibling is not more susceptible to Ewing sarcoma than another.

The cause of Ewing sarcoma is genetic — in that it comes from a person’s genes — but it is not inherited like some other childhood cancers. Parents can’t pass along Ewing sarcoma to their children. The child’s own biological makeup and prenatal development will determine if he or she will develop Ewing sarcoma.

Symptoms of Ewing sarcoma

Common symptoms of Ewing sarcoma include:

Diagnosing Ewing sarcoma

Prompt diagnosis and identification of Ewing sarcoma as early as possible is crucial to overall treatment.

Diagnostic evaluation begins with a thorough medical history and physical examination of your child. A detailed neurological assessment may also be performed. This involves a series of questions and tests to check the brain, spinal cord and nerve function.

At The Children’s Hospital of Philadelphia, clinical experts use a variety of diagnostic tests to diagnose tumors, including:

In addition to diagnosing the specific type of cancer, these tests will also help determine the size, location and stage of the cancer. All of this information is crucial in determining the best treatment option for your child.

Treating Ewing sarcoma

At The Children’s Hospital of Philadelphia, we practice collaborative, family-centered care. A team of expert clinicians — including leading orthopedic surgeons and neurosurgeons, oncologists, advanced practice nurses and physician assistants, pediatric nurses, physical and occupational therapists, psychologists and other specialists — will partner with you in the care of your child.

Our musculoskeletal tumor team is led by orthopedic surgeon John P. Dormans, MD, FACS, chief of the Division of Orthopedic Surgery, who is world renowned for his treatment of childhood cancers, use of limb sparing surgery and clinical research into causes, treatment and long-term outcomes for malignant musculoskeletal tumors.

Treatment options for Ewing sarcoma 

Unlike benign musculoskeletal tumors that may only require “watchful waiting” to see if pain or dysfunction develops, malignant tumors require more active treatment. 

Chemotherapy

Treatment for Ewing sarcoma, a particularly aggressive form of cancer, always involves chemotherapy before and after surgery to kill the main tumor and kill cancer cells that may have already spread, but have not yet been detected.

Chemotherapy refers to medicines that help fight cancer. They are given by mouth, in the vein, in the muscle or under the skin. 

Surgery

Surgical treatment for Ewing sarcoma may be used conjunction with chemotherapy, radiation therapy or proton therapy. 

Surgeries performed are typically:

In a wide resection with margins, the tumor is surgically removed, as well as some of the healthy tissue that surrounded the tumor — the margins of the tumor. In some cases, the child will need reconstructive surgery after the tumor has been cut out.

Reconstructive surgery

Reconstruction can include:

Limb-sparing surgery 

Surgical orthopedic and oncology specialists at CHOP are equipped to perform complex, limb-sparing surgeries for children with cancers like Ewing's sarcoma. CHOP surgeons are constantly pushing the envelope to improve upon existing surgical techniques, and improve outcomes for patients with even the most difficult-to-treat tumors. Learn more about limb-sparing surgery in the video below.

VIDEO APPEARS HERE
 

Surgical safety

Though surgery for malignant tumors is highly effective, we understand that any surgery can be a stressful experience for children and families. At CHOP, we offer a wealth of resources about how to prepare your child for surgery and what to expect during surgery.

Additionally, we employ numerous best practices before, during and after surgery to decrease the risk of infection and increase positive outcomes. For more details about safety protocols at The Children's Hospital of Philadelphia, see safety in surgery

Radiation therapy

Depending on your child’s individual situation, radiation therapy may an option to treat Ewing sarcoma. Radiation therapy uses high-energy waves such as X-rays to kill or shrink cancer cells. It is sometimes used to treat cancer in the central nervous system or other places such as the eye or the testes.

Proton therapy

Proton therapy is an innovative form of radiation treatment that allows for more precise radiation doses delivered to cancerous tumors. Proton therapy’s greatest benefit is that it is less damaging to the surrounding healthy tissue because it delivers most of its energy to a very narrow field at the location of the tumor. Learn more about proton therapy.

Follow-up care for Ewing sarcoma

Your child will see the orthopedic surgeon about one to two weeks after surgery, then again at three and six months post-surgery. Annual monitoring by trained clinicians is strongly encouraged to monitor for possible recurrence of the growth and manage any side effects of treatment.

Ewing sarcoma can reoccur — even after being successfully treated — so it is important for your child to see a physician regularly, especially if any symptoms reoccur.

During follow-up visits, X-rays and other diagnostic testing of the tumor site are recommended to closely monitor your child’s health and ensure there are no side effects from surgery, chemotherapy or proton therapy.

Follow-up care and ongoing support and services are available at our Main Campus and throughout our CHOP Care Network. Our team is committed to partnering with parents and referring physicians to provide the most current, comprehensive and specialized care possible for your child.

Long-term outlook for Ewing sarcoma

The Children's Hospital of Philadelphia has an excellent track record of improving the quality of life for children diagnosed with Ewing sarcoma.

As with any cancer, prognosis and long-term survival can vary greatly from child to child, however. Prompt medical attention and aggressive therapy are important for the best prognosis. 

Resources

For more information on Ewing sarcoma, malignant musculoskeletal tumors and treatment options, visit:

Contact us

To make an appointment with the Division of Orthopedic Surgery at The Children's Hospital of Philadelphia, call 215-590-1527 or contact us online.

 

Reviewed by: John P. Dormans, MD, FACS, and Jason L. Katz, PA-C, MHS, ATC-R
Date: May 2013

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Working Together
to Help Your Child

CHOP's Division of Orthopedic Surgery works closely with the Cancer Center to diagnose and treat children with benign and malignant tumors. Learn more about the Cancer Center at CHOP.

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