Ewing's Sarcoma
all information

Ewing's Sarcoma

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

Pronounced: YOO-ingz sar-KOH-muh

En Español (Spanish Version)

Definition

Ewing's sarcoma is a cancerous bone tumor that may occur in any bone in the body and also in soft tissue (the latter of which is termed an extraosseous sarcoma). The most commonly affected areas include the pelvis, thigh, lower leg, upper arm, and rib.

The cancer most often strikes children and young adults, with puberty being the most common age of onset. During adolescence, Ewing's sarcoma is found equally in boys and girls. In post-adolescent onset, the cancer is more common in males.

Ewing's sarcoma occurs in approximately one in every 50,000 teenagers, accounting for around 30% of all bone cancers that occur in children. Prognosis is dependent upon the location of the tumor and whether the tumor has spread to other areas of the body.

Leg and Pelvic Bones—Common Sarcoma Sites

Leg bones

© 2008 Nucleus Medical Art, Inc.

Causes

The causes of Ewing's sarcoma are not fully understood. Some potential contributing factors include:

  • Chromosomal rearrangement that alters the genes (fusion of the EWS gene to specific transcription factor genes)
  • Injury or trauma to the bone
  • Prior high-dose radiation exposure

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition. Because the causes of the cancer are unknown, risk factors are also not fully understood. The following factors may increase your chance of developing Ewing's sarcoma:

  • Having another congenital disease
  • Having had another malignancy, particularly one treated with radiotherapy

Ewing's sarcoma is not an inherited condition. Having family members who have the cancer does not increase the likelihood of developing the cancer. Also, people who are of African American or Asian descent are at a significantly lower risk of developing Ewing's sarcoma than Caucasians.

Symptoms

Symptoms include:

  • Pain, redness, and swelling surrounding the tumor
  • Fever
  • Weight loss and reduced appetite
  • Fatigue
  • Paralysis and incontinence (in cases where the tumor is near the spinal region)
  • Numbness, tingling, and paralysis (caused by compression of the nerves by the tumor)

Diagnosis

Your doctor will ask about your symptoms and medical history, and perform a physical exam. You may need to see an oncologist, a doctor who specializes in cancer.

Tests may include the following:

  • Bone scans —to detect presence of a tumor
  • CT scan —a type of x-ray that uses a computer to look for cancer that has spread to the lungs
  • MRI scan —a test that uses magnetic waves to make pictures of structures inside the body
  • PET scan —to evaluate the metabolic activity of tissue
  • Blood tests—to determine abnormalities in the blood
  • Biopsy —to determine if a tumor is malignant

Bone Biopsy

Bone biopsy

© 2008 Nucleus Medical Art, Inc.

Treatment

Talk with your doctor about the best treatment plan for you. Treatment options include:

Surgery

Surgery may be used to remove the tumor.

Radiation Therapy

High-energy x-rays may be used to kill tumor cells.

Chemotherapy

Chemotherapy drugs may be used to kill tumor cells. Targeted chemotherapies focused on the rearranged genes and their products in Ewing’s sarcoma are being actively investigated. Targets located “downstream” from the relevant pathways in Ewing’s sarcoma are also serving as potential targets.

Myeloablative Therapy

An intense dose of chemotherapy treatment may be used for patients who have been resistant to other treatment methods.

Prevention

Ewing's Sarcoma occurs for unknown reasons. There are currently no preventive measures to reduce the risk of developing the cancer.

RESOURCES:

Cancer Index
http://www.cancerindex.org/

US National Library of Medicine
http://www.nlm.nih.gov/

CANADIAN RESOURCES:

About Kids Health
http://www.aboutkidshealth.ca

Alberta Children's Services
http://www.child.alberta.ca/home/

References:

DynaMed website. Available at: http://dynamed102.ebscohost.com/Detail.aspx?id=114929 .

Ewing's sarcoma. Bone Disorders. University of Utah Health Sciences Center website. Available at: http://healthcare.utah.edu/healthinfo/adult/bone/ewings.htm . Accessed June 24, 2007.

Ewing’s Sarcoma. Mayo Clinic. Available at: http://www.mayoclinic.org/ewings-sarcoma/

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. Pediatric Blood Cancer . 2007;49:894-900.

Sarcomas: Ewing's sarcoma. The University of California, San Francisco Children's Hospital website. Available at: http://www.ucsfhealth.org/childrens/medical_services/cancer/sarcomas/conditions/ewings/signs.html . Accessed June 24, 2007.

Solid tumors: Ewing sarcoma family tumors. Disease Information. St. Jude Children's Research Hospital website. Available at: http://www.stjude.org/disease-summaries/0,2557,449_2167_2956,00.html . Accessed June 26, 2007.



Last reviewed April 2008 by Rimas Lukas, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.


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