This MedLibrary.org supplementary page on Ewing's sarcoma is provided directly from the open source Wikipedia as a service to our readers. Please see the note below on authorship of this content, as well as the Wikipedia usage guidelines. To search for other content from our encyclopedia supplement, please use the form below:
Related Sponsors
| Ewing sarcoma Classification and external resources |
|
 |
|
|---|---|
| X-Ray of a child with Ewing sarcoma of the tibia | |
| ICD-9 | 170.9 |
| ICD-O: | 9260/3 |
| OMIM | 133450 |
| DiseasesDB | 4604 |
| MedlinePlus | 001302 |
| eMedicine | ped/2589 |
| MeSH | D012512 |
Ewing sarcoma is a malignant round-cell tumor. It is a rare disease in which cancer cells are found in the bone or in soft tissue. The most common areas in which it occurs are the pelvis, the femur, the humerus, and the ribs.
Because a common genetic locus is responsible for a large percentage of Ewing sarcoma and primitive neuroectodermal tumors, these are sometimes grouped together in a category known as the Ewing family of tumors.1 The diseases are, however, considered to be different: peripheral primitive neuroectodermal tumors are generally not associated with bones, while Ewing sarcomas are most commonly related to bone.
Ewing sarcoma occurs most frequently in male teenagers, with a male/female ratio of 1.6:1.2
Although usually classified as a bone tumor, Ewing sarcoma can have characteristics of both mesodermal and ectodermal origin, making it difficult to classify.3
Contents |
Eponym
James Ewing (1866-1943) first described the tumor, establishing that the disease was separate from lymphoma and other types of cancer known at that time. 45
Causes
Ewing sarcoma is the result of a translocation between chromosomes 11 and 22, which fuses the EWS gene of chromosome 22 to the FLI1 gene of chromosome 11.
EWS/FLI functions as the master regulator.6
Clinical findings
Ewing sarcoma is more common in males and usually presents in childhood or early adulthood, with a peak between 10 and 20 years of age. It can occur anywhere in the body, but most commonly in the pelvis and proximal long tubular bones. The diaphyses of the femur are the most common sites, followed by the tibia and the humerus. Thirty percent are overtly metastatic at presentation.
It is positive for CD99 and negative for CD45.7
Imaging findings
On conventional radiographs, the most common osseous presentation is a permeative lytic lesion with periosteal reaction. The classic description of lamellated or "onion skin" type periosteal reaction is often associated with this lesion. Plain films add valuable information in the initial evaluation or screening. The wide zone of transition (e.g. permeative) is the most useful plain film characteristic in differentiation of benign versus aggressive or malignant lytic lesions.
MRI should be routinely used in the work-up of malignant tumors. MRI will show the full bony and soft tissue extent and relate the tumor to other nearby anatomic structures (e.g. vessels). Gadolinium contrast is not necessary as it does not give additional information over noncontrast studies, though some current researchers argue that dynamic, contrast enhanced MRI may help determine the amount of necrosis within the tumor, thus help in determining response to treatment prior to surgery.
CT can also be used to define the extraosseous extent of the tumor, especially in the skull, spine, ribs and pelvis. Both CT and MRI can be used to follow response to radiation and/or chemotherapy.
Bone scintigraphy can also be used to follow tumor response to therapy.
Differential diagnosis
Other entities that may have a similar clinical presentation include osteomyelitis, osteosarcoma (especially telangiectatic osteosarcoma) and eosinophilic granuloma. Soft tissue neoplasms such as malignant fibrous histiocytoma that erode into adjacent bone may also have a similar appearance.
Epidemiology
The frequency in the United States depends on the patient's age, with a rate of 0.3 case per 1,000,000 children in those younger than 3 years of age to as high as 4.6 cases per 1,000,000 in adolescents aged 15-19 years. Internationally the annual incidence rate averages less than 2 cases per 1,000,000 children.8 In the United Kingdom an average of six children per year are diagnosed, mainly males in early stages of puberty. Due to the prevalence of diagnosis during teenage years, there may possibly be a link between the onset of puberty and the early stages of this disease, although no research is currently being conducted to confirm this theory.
Treatment
Because almost all patients with apparently localized disease at diagnosis have occult metastatic disease, multidrug chemotherapy (often including ifosfamide and etoposide)9 as well as local disease control with surgery and/or radiation is indicated in the treatment of all patients.citation needed
Treatment often consists of neo-adjuvant chemotherapy generally followed by wide or radical excision, and may also include radiotherapy. Complete excision at the time of biopsy may be performed if malignancy is confirmed at that time. Treatment lengths vary depending on location and stage of the disease at diagnosis. Radical chemotherapy may be as short as 6 treatments at 3 week cycles, however most patients will undergo chemotherapy for 6-12 months and radiation therapy for 5-8 weeks.
Antisense oligodeoxynucleotides have been proposed as possible treatment by down-regulating the expression of the oncogenic fusion protein associated with the development of Ewing sarcoma resulting from the EWS-ETS gene translocation.1011In addition, the synthetic retinoid derivative fenretinide (4-hydroxy(phenyl)retinamide) has been reported to induce high levels of cell death in Ewing sarcoma cell lines in vitro and to delay growth of Ewing sarcoma xenografts in vivo mouse models. 1213
Prognosis
Staging attempts to distinguish patients with localized from those with metastatic disease.14 Most commonly, metastases occur in the chest, bone and/or bone marrow. Less common sites include the central nervous system and lymph nodes.
Survival for localized disease is 70% to 80% when treated with chemotherapy.15 Long term survival for metastatic disease can be less than 10% but some sources state it is 25-30%.citation needed.
References
- ^ Iwamoto Y (February 2007). "Diagnosis and treatment of Ewing sarcoma". Jpn. J. Clin. Oncol. 37 (2): 79–89. doi:. PMID 17272319. http://jjco.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=17272319.
- ^ Burt M, Karpeh M, Ukoha O, et al (January 1993). "Medical tumors of the chest wall. Solitary plasmacytoma and Ewing sarcoma". J. Thorac. Cardiovasc. Surg. 105 (1): 89–96. PMID 8419714. http://jtcs.ctsnetjournals.org/cgi/content/abstract/105/1/89.
- ^ Longtin R (November 2003). "Ewing sarcoma: a miracle drug waiting to happen?". J. Natl. Cancer Inst. 95 (21): 1574–6. PMID 14600088. http://jnci.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=14600088.
- ^ synd/2367 at Who Named It
- ^ J. Ewing. Diffuse endothelioma of bone. Proceedings of the New York Pathological Society, 1921, 17-24.
- ^ Owen LA, Kowalewski AA, Lessnick SL (2008). "EWS/FLI mediates transcriptional repression via NKX2.2 during oncogenic transformation in Ewing sarcoma". PLoS ONE 3 (4): e1965. doi:. PMID 18414662. http://www.plosone.org/article/info:doi/10.1371/journal.pone.0001965.
- ^ Bernstein M, Kovar H, Paulussen M, et al (May 2006). "Ewing sarcoma family of tumors: current management". Oncologist 11 (5): 503–19. doi:. PMID 16720851. http://theoncologist.alphamedpress.org/cgi/pmidlookup?view=long&pmid=16720851.
- ^ http://www.emedicine.com/radio/topic275.htm eMedicine article on Ewing sarcoma accessed April 24, 2006
- ^ Lahl M, Fisher VL, Laschinger K (February 2008). "Ewing sarcoma family of tumors: an overview from diagnosis to survivorship". Clin J Oncol Nurs 12 (1): 89–97. doi:. PMID 18258578. http://ons.metapress.com/openurl.asp?genre=article&doi=10.1188/08.CJON.89-97.
- ^ Asami S, Chin M, Shichino H, et al (March 2008). "Treatment of Ewing sarcoma using an antisense oligodeoxynucleotide to regulate the cell cycle" (dead link – Scholar search). Biol. Pharm. Bull. 31 (3): 391–4. PMID 18310898. http://joi.jlc.jst.go.jp/JST.JSTAGE/bpb/31.391?from=PubMed.
- ^ Mateo-Lozano S, Gokhale PC, Soldatenkov VA, Dritschilo A, Tirado OM, Notario V (November 2006). "Combined transcriptional and translational targeting of EWS/FLI-1 in Ewing sarcoma". Clin. Cancer Res. 12 (22): 6781–90. doi:. PMID 17121899. http://clincancerres.aacrjournals.org/cgi/pmidlookup?view=long&pmid=17121899.
- ^ Myatt SS, Redfern CP, Burchill SA. p38MAPK-Dependent sensitivity of Ewing sarcoma family of tumors to fenretinide-induced cell death.Clin Cancer Res. 2005 Apr 15;11(8):3136-48.
- ^ Myatt SS, Burchill SA. The sensitivity of the Ewing sarcoma family of tumours to fenretinide-induced cell death is increased by EWS-Fli1-dependent modulation of p38(MAPK) activity.Oncogene. 2008 Feb 7;27(7):985-96.
- ^ McTiernan AM, Cassoni AM, Driver D, Michelagnoli MP, Kilby AM, Whelan JS (2006). "Improving Outcomes After Relapse in Ewing Sarcoma: Analysis of 114 Patients From a Single Institution". Sarcoma 2006: 83548. doi:. PMID 17496997.
- ^ "ACS :: How Is the Ewing Family of Tumors Staged?".
External links
|
||||||||||||||||||||||
|
||||||||||||||
|
|||||||||||||||||||||||||||
Wikipedia content modification information:
- This page was last modified on 3 January 2009, at 23:26.
Wikipedia Authorship and Review
Wikipedia content provided here is not reviewed directly by MedLibrary.org. Wikipedia content is authored by an open community of volunteers and is not produced by or in any way affiliated with MedLibrary.org.
Wikipedia Usage Guidelines
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article on "Ewing's sarcoma".
The URL for this specific entry is:
All Wikipedia text is available under the terms of the GNU Free Documentation License. (See Copyrights for details). Wikipedia® is a registered trademark of the Wikimedia Foundation, Inc.