What are the other Names for this Condition? (Also known as/Synonyms)
- EFT (Ewing's Family of Tumors)
What is Ewing's Family of Tumors? (Definition/Background Information)
Ewing’s Family of Tumors (EFT) 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 Family of Tumors are a group of life-threatening and malignant type of tumors that affect both children and adults
- It is believed that these tumors are 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 EFTs 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 Family of Tumors? (Age and Sex Distribution)
- Both children and adults are affected by Ewing’s Family of Tumors; however, predominantly EFT affects young children. This condition is rare in adults over 30 years, and extremely infrequent in adults, over 45 years of age
- Males are generally affected more than females, although this depends primarily on the tumor subtype
- Caucasians are more prone to EFTs, much more than any other race or ethnic group
What are the Risk Factors for Ewing's Family of Tumors? (Predisposing Factors)
- The risk factor depends on the subtype of EFT, an individual is affected with
- Certain genetic mutations or genetic conditions may be possible causal factors for Ewing’s Family of Tumors
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 Family of Tumors? (Etiology)
- The cause of development of Ewing’s Family of Tumors is currently unknown
- Some types 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 Family of Tumors?
Ewing’s Family of Tumors presentations are based on the location of the tumor. The locations could be the bone, brain, skin, chest wall, and soft tissues of the body. A few signs and symptoms of EFT may include:
- 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
- Weight loss, changes in appetite
- Bone destruction (moth-eaten, permeative), may result in bones breaking easily, at the site of the tumor
- Morning nausea and vomiting
- Morning headaches that gradually improve as the day progresses
- Personality or behavioral changes
How are Ewing's Family of Tumors Diagnosed?
Diagnostic tools for Ewing’s Family of Tumors include:
- Physical exam with complete medical history evaluation
- MRI, CT scans of the affected regions, to examine magnitude of the tumor mass and its spread
- Neurological exam, if tumor is in the brain: Test of all brain and spinal functions
- 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 EFT subtype
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
- Mesenchymal chondrosarcoma
- 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 Family of Tumors?
Complications due to Ewing’s Family of Tumors are:
- 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
- Severe conditions may develop, if the brain is affected
How are Ewing's Family of Tumors Treated?
Treatment measures for Ewing’s Family of Tumors include the following:
- The treatment protocol that is planned by a team of healthcare professionals is based on a variety of factors, such as:
- EFT tumor subtype
- 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
How can Ewing's Family of Tumors be Prevented?
- Current medical research have not established a way of preventing Ewing’s Family of Tumors
- 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 any type of EFT
- Besides, due to their high metastasizing potential, often, several years of active vigilance is necessary
What is the Prognosis of Ewing's Family of Tumors? (Outcomes/Resolutions)
- The prognosis for individuals with Ewing’s Family of Tumors is dependent on several factors. These include:
- EFT subtype and grade
- Age and health status of the individual, at the time of diagnosis
- Size and location of the tumor
- If the tumor can be removed through surgery and the amount of tumor mass that can be safely removed
- Whether the tumor has recurred, or has spread to other areas of the body
- The prognosis is better if the tumor 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
- Improved outcomes have been obtained with radiotherapy and chemotherapy and the survival rate is above 40%
Additional and Relevant Useful Information for Ewing's Family of Tumors:
- Ewing’s Family of Tumors 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:
What are some Useful Resources for Additional Information?
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126 Gaithersburg, MD 20898-8126
Toll-Free: (888) 205-2311
TTY: (888) 205-3223
International Telephone Access Number: (301) 251-4925
Fax: (301) 251-4911
The University of Texas MD Anderson Cancer Center
1515 Holcombe Blvd, Houston, TX 77030
Phone: (713) 792-2121
askMDAnderson: 1 (877) 632-6789
References and Information Sources used for the Article:
http://www.cancer.net/cancer-types/ewing-family-tumors-childhood (accessed on 01/30/14)
http://www.cancer.org/acs/groups/cid/documents/webcontent/003099-pdf.pdf (accessed on 01/30/14)
http://www.ncbi.nlm.nih.gov/pubmed/15939194 (accessed on 01/30/14)
http://jco.ascopubs.org/content/17/1/180.full (accessed on 01/30/14)
Pathology & Genetics: Tumours of Soft Tissue and Bone; WHO Classification of Tumours; Edited by Christopher D.M. Fletcher, K. Krishnan Unni, Fredrik Mertens; IARC Press, Lyon, 2002
Helpful Peer-Reviewed Medical Articles:
Schmidt D, Herrmann C, Jurgens H, et al: Malignant peripheral neuroectodermal tumor and its necessary distinction from Ewing's sarcoma. A report from the Kiel Pediatric Tumor Registry. Cancer 68:2251-2259, 1991
Hijazi Y, Worthy E, Steinberg S, et al: Ewing's sarcoma (ES) vs. primitive neuroectodermal tumor (PNET): Does histology play a role in prognosis. Mod Pathol 6:126A, 1993
Ewing's sarcoma and primitive neuroectodermal family of tumors. Carvajal R, Meyers P. Hematol Oncol Clin North Am. 2005 Jun; 19(3):501-25, vi-vii.
Ewing's sarcoma and peripheral primitive neuroectodermal tumor in adults: different features of a rare neoplasm. Armbruster C, Huber M, Prosch H, Dworan N, Attems J. Onkologie. 2008 Apr; 31(4):179-84. Epub 2008 Mar 20.
Kang, M. H., Wang, J., Makena, M. R., Lee, J. S., Paz, N., Hall, C. P., ... & Ko, W. (2015). Activity of MM-398, Nanoliposomal Irinotecan (nal-IRI), in Ewing's Family Tumor Xenografts Is Associated with High Exposure of Tumor to Drug and High SLFN11 Expression. Clinical Cancer Research, 21(5), 1139-1150.
Luber, B. S., Leal, J. P., Wang, H., Bolejack, V., Schuetze, S. M., Schwartz, L., ... & Wahl, R. L. (2016). Response to early treatment evaluated with 18F‐FDG PET and PERCIST 1.0 predicts survival in patients with Ewing sarcoma family of tumors treated with a monoclonal antibody to the insulin‐like growth factor 1 receptor. Journal of Nuclear Medicine, jnumed-115.
Biswas, B., Thakar, A., Mohanti, B. K., Vishnubhatla, S., & Bakhshi, S. (2015). Prognostic factors in head and neck Ewing sarcoma family of tumors. The Laryngoscope, 125(3), E112-E117.
Yun, T., Suzuki, H., Mizobuchi, T., Sakairi, Y., Nagato, K., Nakajima, T., ... & Yoshino, I. (2015). Late recurrence of a tumor of Ewing’s sarcoma family of tumors: report of a case. Surgical Case Reports, 1(1), 1.
Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Feb. 2, 2014
Last updated: Feb. 25, 2017
Was this article helpful?