Ewing Sarcoma

(Peripheral Primitive Neuroectodermal Tumors [PNET]; Ewing Family of Tumors)

Pronounced: YOO-ingz sar-KOH-muh

Definition

Ewing sarcoma is a type of bone cancer. The most commonly affected areas include the pelvis, thigh, lower leg, upper arm, and chest wall.

Leg and Pelvic Bones—Common Sarcoma Sites

Leg bones
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Causes    TOP

Cancer is an uncontrolled division of cells. These cells group together and form a tumor. They damage tissue nearby and make it difficult for healthy cells to work as they should.

Ewing sarcoma often starts because of a change in chromosomes. It is not clear why these changes happen.

Risk Factors    TOP

Risk factors are not clear since the cause is unknown. This type of cancer is more common in male teenagers.

Symptoms    TOP

Symptoms will depend on where the tumor is. General symptoms may include:

  • Pain which:
    • May come and go or be constant
    • Range from mild to severe
    • May include feeling of pins and needles
  • Redness and swelling around the area
  • Fatigue
  • Fever
  • Weight loss and reduced appetite
  • Broken bone with no known cause

Diagnosis    TOP

The doctor will ask about your symptoms and health history. A physical exam will also be done. There are a number of bone issues that cause the same symptoms. Tests will help to confirm ewing sarcoma.

The doctor will do a biopsy. A sample of tissue will be removed from the area. A lab will look for cancer cells.

Bone Biopsy

Bone biopsy
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Image tests will show the size and location of the tumor. Tests may include:

The doctor may also order blood tests. It will help to show if bone tissue was damaged.

Treatment    TOP

The goal of treatment is to remove or kill as much of the cancer as possible. Treatment will be based on tumor size and location but often includes a combination of:

Chemotherapy

Chemotherapy drugs are used to kill tumor cells. Your doctor will prescribe a combination of drugs to be given over a set time. This usually means every 2-4 weeks for several months.

This is often the first step in treatment. It may help to shrink the tumor before other treatment. It can also attack tumors that may have spread to other areas of the body.

Surgery

The tumor may be removed with surgery. It is often done after chemotherapy to try to shrink the tumor first.

The bone may need repair after the tumor is removed. A bone graft or prosthesis may be needed to support the remaining bone. It may take a number of surgeries to get full use of the limb.

Radiation Therapy    TOP

Radiation therapy uses high-energy x-rays to kill tumor cells. It can help shrink tumors before a surgery. It may also be used instead of surgery. It is often given over several weeks.

Future Therapy    TOP

A high-dose chemotherapy is being tested for ewing sarcoma. This high dose kills off bone marrow. A stem cell transplant is then needed to help grow healthy bone marrow. This combination therapy is typically used in cases where the tumor is resistant.

Prevention    TOP

There are no known steps for prevention since the cause is not clear.

RESOURCES:

American Cancer Society
http://www.cancer.org
National Cancer Institute
http://www.cancer.gov

CANADIAN RESOURCES:

Canadian Cancer Society
http://www.cancer.ca
Ewings Cancer Foundation of Canada
http://ewingscancer.ca

References:

Ewing family of tumors. American Cancer Society website. Available at:
...(Click grey area to select URL)
Accessed September 6, 2016.
Ewing sarcoma. EBSCO DynaMed Plus website. Available at: Available at:
...(Click grey area to select URL)
Updated October 29, 2014. Accessed September 28, 2016.
Ewing sarcoma. St. Jude Children's Research Hospital website. Available at: https://www.stjude.org/disease/ewing-sarcoma.html. Accessed September 6, 2016.
Ewing’s sarcoma. Ortho Info—American Academy of Orthopaedic Surgeons website. Available at:
...(Click grey area to select URL)
Updated September 2011. Accessed September 6, 2016.
Ewing's sarcoma. UCSF Benioff Children's Hospital website. Available at: https://www.ucsfbenioffchildrens.org/conditions/ewings_sarcoma/index.html. Accessed September 6, 2016.
Miser ES, Goldsby RE, et al. Treatment of metastatic Ewing’s sarcoma/primitive neuroectodermal tumor of bone: evaluation of increasing the dose intensity of chemotherapy-a report from the children’s oncology group. Pediatr Blood Cancer. 2007;49(7):894-900.
Last reviewed May 2018 by Mohei Abouzied, MD
Last Updated: 5/8/2018

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