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Ewing Sarcoma

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

Pronounced: YOO-ingz sar-KOH-muh

by Michael Jubinville, MPH

Home | Health Information Center | Disease, Condition, & Injury Fact Sheets | Article

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

Causes

Cancer is when cells in the body split without control or order. These cells go on to form a growth or tumor. The term cancer refers to harmful growths. These growths attack nearby tissues. They also spread to other parts of the body. It’s not clear exactly what causes these problems. It’s likely a mix of genes and the environment.

Risk Factors

Risk factors are not clear since the cause is unknown. This type of cancer is more common in male teenagers who are White. But it can happen at any age.

Symptoms

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

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

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

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
Surgery
Radiation

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

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

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

There is no way to prevent Ewing sarcoma since the cause is unknown.

RESOURCES:

American Cancer Society
https://www.cancer.org

National Cancer Institute
https://www.cancer.gov

CANADIAN RESOURCES:

Canadian Cancer Society
https://www.cancer.ca

Ewings Cancer Foundation of Canada
http://ewingscancer.ca

References:

Ewing family of tumors. American Cancer Society website. Available at: https://www.cancer.org/cancer/ewing-tumor.html. Accessed January 29, 2021.

Ewing sarcoma treatment (PDQ)—patient version. National Cancer Institute website. Available at: https://www.cancer.gov/types/bone/patient/ewing-treatment-pdq. Accessed January 29, 2021.

Ewing’s sarcoma. Ortho Info—American Academy of Orthopaedic Surgeons website. Available at: https://orthoinfo.aaos.org/en/diseases--conditions/ewings-sarcoma. Accessed January 29, 2021.

Miser ES, Goldsby RE, Chen Z, 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 September 2020 by EBSCO Medical Review Board Mohei Abouzied, MD, FACP
Last Updated: 1/29/2021

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