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Ewing’s Sarcoma of Bone

Last updated Sept. 11, 2018

Approved by: Maulik P. Purohit MD, MPH


Microscopic pathology image showing Ewing's sarcoma of bone (H and E stain).

What are the other Names for this Condition? (Also known as/Synonyms)

  • ES of Bone (Ewing’s Sarcoma of Bone)
  • Ewing Tumor of Bone
  • Ewing’s Sarcoma-Primitive Neuroectodermal Tumor of Bone

What is Ewing’s Sarcoma of Bone? (Definition/Background Information)

Ewing’s Family of Tumors includes the following tumors:

  • Ewing’s Sarcoma of Bone
  • Extraosseous Ewing’s Tumor
  • Primitive Neuroectodermal Tumor (PNET), including Medulloblastoma - Adult type and Medulloblastoma - Childhood type
  • Askin’s Tumor: This tumor is also sometimes called, Primitive Neuroectodermal Tumor of Chest Wall, and Ewing’s Sarcoma of Chest Wall
  • Primary Cutaneous Ewing’s Sarcoma (PCES)

All the above-mentioned tumors arise from a primitive stem cell.

  • Ewing’s Sarcoma of Bone (or ES of Bone) is a life-threatening and malignant type of bone tumor that affects young children
  • The long bones of the body, such as the ribs, femur, and tibia, are the usual sites for ES of Bone; but it can develop in any bone, anywhere on the body, including the pelvis. However, it is not commonly observed in the bones of the skull, vertebra, short bones of the hands and legs
  • It is believed that the tumor is caused by genetic defects on chromosome 11, chromosome 22, chromosome 8, and chromosome 12
  • However, with a global incidence rate of less than 1 in 500,000, Ewing’s Family of Tumors are rare types of tumors
  • The treatment modalities used to treat this tumor are surgery, chemotherapy, and radiation therapy. The prognosis depends on the stage of the tumor; metastatic tumors have a poor prognosis

Who gets Ewing’s Sarcoma of Bone? (Age and Sex Distribution)

  • Children and young adults 5-20 years old are primarily affected by Ewing’s Sarcoma of Bone (in about 80% of the cases). ES of Bone is the second most common bone cancer in children and adolescents
  • Adults over the age of 30 years, are highly unlikely to get this condition
  • Boys are affected more commonly than girls, in a 7:5 ratio
  • Caucasians are more prone to ES of Bone, than Asians, Africans, and Afro-Americans, by a factor of 10. Hence, ES of Bone is rarely seen in African Americans and Asians

What are the Risk Factors for Ewing’s Sarcoma of Bone? (Predisposing Factors)

  • Genetic mutations could be a possible causal factor for Ewing’s Sarcoma of Bone
  • Prior radiation therapy for a completely different tumor may cause the development of ES of Bone, at the site of radiation therapy

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Ewing’s Sarcoma of Bone? (Etiology)

  • Ewing’s Sarcoma of Bone display recurrent chromosomal translocation (between chromosomes 11 and 22) with minor chromosomal anomalies (on chromosomes 8 and 12)
  • Specific translocations between chromosomes 11 and 22 is present, in over 85% of the patients
  • Such genetic mutations are known to have potentially cancerous traits. However, a specific reasons for these mutations to occur, is not established yet

What are the Signs and Symptoms of Ewing’s Sarcoma of Bone?

The presentations are based on the location of the bone involved by the tumor. Signs and symptoms of Ewing’s Sarcoma of Bone may include:

  • This bone tumor usually originate in the diaphysis (or metaphyseal-diaphyseal) region of long bones
  • Fever, which may be observed frequently, may indicate an infection
  • Anemia (low red cell count in blood); leukocytosis (above normal levels of white blood cells)
  • Increased blood erythrocyte sedimentation rate
  • Bone destruction (moth-eaten, permeative), may result in bones breaking easily, at the site of the tumor

How is Ewing’s Sarcoma of Bone Diagnosed?

Diagnostic tools for Ewing’s Sarcoma of Bone include:

  • Physical exam with complete medical history evaluation
  • X-rays of the affected bones - the affected bones may show osteolytic lesion
  • MRI scans, CT scans, to examine magnitude of the tumor mass and its spread
  • Biopsy of tumor - the specimen is examined under a microscope by a pathologist, to arrive at a definitive diagnosis
  • Genetic tests and analysis, to test for specific mutations associated with ES of Bone

Differential diagnosis, to eliminate the following tumor types may be considered, before arriving at a definitive diagnosis:

  • Dedifferentiated synovial sarcoma
  • Desmoplastic small round cell tumors
  • Lymphoma
  • Medulloblastoma
  • Mesenchymal chondrosarcoma
  • Rhabdomyosarcoma
  • Small cell osteosarcoma

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Ewing’s Sarcoma of Bone?

Complications due to Ewing’s Sarcoma of Bone are:

  • Pathological fracture (which may occur in rare cases)
  • Metastasis of the tumor to other vital body organs, such as the lungs and bone marrow
  • Damage of the bones, vital nerves, and blood vessels, during surgery
  • Side effects from chemotherapy (such as toxicity), radiation therapy
  • Recurrence of the tumor after treatment

How is Ewing’s Sarcoma of Bone Treated?

Treatment measures for Ewing’s Sarcoma of Bone include the following:

  • The treatment protocol that is planned by a team of healthcare professionals is based on a variety of factors, such as:
    • The tumor stage
    • Location of the tumor
    • Tumor size
    • And whether the tumor has metastasized
  • Any combination of chemotherapy, radiation therapy and invasive procedures are used to treat this tumor. Generally, the treatment is administered at a medical center that has prior experience in treating such tumors
  • Autologous bone marrow transplant and stem cell transplantation are also used, depending on the clinical situation of the particular patient

How can Ewing’s Sarcoma of Bone be Prevented?

  • Current medical research have not established a way of preventing Ewing’s Sarcoma of Bone
  • Genetic counseling and genetic testing could help those individuals having a family history of the condition, planning for a child
  • Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already endured ES of Bone
  • Besides, due to its high metastasizing potential, often, several years of active vigilance is necessary

What is the Prognosis of Ewing’s Sarcoma of Bone? (Outcomes/Resolutions)

  • The prognosis is better if Ewing’s Sarcoma of Bone is:
    • Diagnosed at an early stage
    • Features a small-sized tumor that has not spread to other areas
    • The individuals’ response to the treatment procedure is good
  • Over 65% of children, who have localized ES of Bone (low-stage tumors), survive for a longer period
  • The tumors that occur in the bones, which are easily accessible to surgery, have a better prognosis, than those tumors affecting bones that are difficult to access for surgery. For example, ES of the tibia bone of leg, is more easily accessible to surgery, than the pelvic bone
  • If the tumor has spread to other parts of the body, then less than one-third of the individuals, survive the condition
  • Improved outcomes have been obtained with radiotherapy and chemotherapy and the survival rate is above 40%

Additional and Relevant Useful Information for Ewing’s Sarcoma of Bone:

  • Ewing’s Sarcoma of Bone and its management can cause physical and emotional distress. Often, supportive care and encouragement help positively in bringing about a measure of relief, to the patients.
  • Both, Ewing’s Sarcoma and Primitive Neuroectodermal Tumor, are round cell sarcomas (types of cancer), with ES lacking neuroectodermal differentiation, while PNET presents such features
  • It is estimated that 200 new cases of Ewing’s Family of Tumors are diagnosed newly in the US per year

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Feb. 2, 2014
Last updated: Sept. 11, 2018