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Ewing sarcoma
Classification and external resources

X-Ray of a child with Ewing sarcoma of the tibia
ICD-9 170.9
ICD-O: M9260/3
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. [4][5]

Causes

Genetic exchange between chromosomes can cause cells to become cancerous, like these cells from metastasized Ewing sarcoma. 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]

Other translocations are at t(21;22)[7] and t(7;22).[8]

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, especially around the growth plates. The diaphyses of the femur are the most common sites, followed by the tibia and the humerus. Thirty percent are overtly metastatic at presentation. Patients usually experience extreme bone pain.

It is positive for CD99 and negative for CD45.[9]

Imaging findings

Magnetic resonance imaging slice showing Ewing's sarcoma of the left hip (white area shown right).

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.[10] 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)[11] as well as local disease control with surgery and/or radiation is indicated in the treatment of all patients [12].

Treatment often consists of neo-adjuvant chemotherapy generally followed by a limb salvage or an amputation and may also include radiotherapy. Complete excision at the time of biopsy may be performed if malignancy is confirmed at the time it is examined. 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.[13][14] In 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. [15][16]

Prognosis

Staging attempts to distinguish patients with localized from those with metastatic disease.[17] Most commonly, metastases occur in the chest, bone and/or bone marrow. Less common sites include the central nervous system and lymph nodes.

Five-year survival for localized disease is 70% to 80% when treated with chemotherapy. [18] Long term survival for metastatic disease can be less than 10% but some sources state it is 25-30%..

References

  1. ^ Iwamoto Y (February 2007). "Diagnosis and treatment of Ewing sarcoma". Jpn. J. Clin. Oncol. 37 (2): 79–89. doi:10.1093/jjco/hyl142. PMID 17272319. http://jjco.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=17272319.  
  2. ^ 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.  
  3. ^ 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.  
  4. ^ synd/2367 at Who Named It?
  5. ^ J. Ewing. Diffuse endothelioma of bone. Proceedings of the New York Pathological Society, 1921, 17-24.
  6. ^ 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:10.1371/journal.pone.0001965. PMID 18414662. http://www.plosone.org/article/info:doi/10.1371/journal.pone.0001965.  
  7. ^ Sorensen PH, Lessnick SL, Lopez-Terrada D, Liu XF, Triche TJ, Denny CT (February 1994). "A second Ewing's sarcoma translocation, t(21;22), fuses the EWS gene to another ETS-family transcription factor, ERG". Nat. Genet. 6 (2): 146–51. doi:10.1038/ng0294-146. PMID 8162068.  
  8. ^ Jeon IS, Davis JN, Braun BS, et al. (March 1995). "A variant Ewing's sarcoma translocation (7;22) fuses the EWS gene to the ETS gene ETV1". Oncogene 10 (6): 1229–34. PMID 7700648.  
  9. ^ Bernstein M, Kovar H, Paulussen M, et al. (May 2006). "Ewing sarcoma family of tumors: current management". Oncologist 11 (5): 503–19. doi:10.1634/theoncologist.11-5-503. PMID 16720851. http://theoncologist.alphamedpress.org/cgi/pmidlookup?view=long&pmid=16720851.  
  10. ^ http://www.emedicine.com/radio/topic275.htm eMedicine article on Ewing sarcoma accessed April 24, 2006
  11. ^ 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:10.1188/08.CJON.89-97. PMID 18258578. http://ons.metapress.com/openurl.asp?genre=article&doi=10.1188/08.CJON.89-97.  
  12. ^ Randall, RL (2005). "Ewing's Sarcoma Family of Tumors (ESFT)". ESUN. http://sarcomahelp.org/learning_center/ewings_sarcoma.html. Retrieved 2009-04-15.  
  13. ^ Asami S, Chin M, Shichino H, et al. (March 2008). "Treatment of Ewing sarcoma using an antisense oligodeoxynucleotide to regulate the cell cycle" ( – Scholar search). Biol. Pharm. Bull. 31 (3): 391–4. doi:10.1248/bpb.31.391. PMID 18310898. http://joi.jlc.jst.go.jp/JST.JSTAGE/bpb/31.391?from=PubMed.  
  14. ^ 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:10.1158/1078-0432.CCR-06-0609. PMID 17121899. http://clincancerres.aacrjournals.org/cgi/pmidlookup?view=long&pmid=17121899.  
  15. ^ 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.
  16. ^ 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.
  17. ^ 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:10.1155/SRCM/2006/83548. PMID 17496997.  
  18. ^ "ACS :: How Is the Ewing Family of Tumors Staged?". http://www.cancer.org/docroot/CRI/content/CRI_2_4_3X_How_is_Ewings_Family_of_tumors_staged_48.asp?sitearea=.  
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Wiktionary

Up to date as of January 15, 2010

Definition from Wiktionary, a free dictionary

Contents

English

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Wikipedia

Etymology

Ewing's from James Ewing + -'s; + sarcoma.

Noun

Singular
Ewing's sarcoma

Plural
uncountable

Ewing's sarcoma (uncountable)

  1. (pathology) A particular type of cancer of the bone.

Anagrams


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