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En Wikipedia Org Wiki Ewing's Sarcoma

En Wikipedia Org Wiki Ewing's Sarcoma

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Published by: nuke1961 on Dec 14, 2011
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Ewing sarcoma
Classification and external resources
 Micrograph of metastatic Ewing sarcoma (right of image) in normal lung (leftof image). PAS stain.
ICD-9
170.9 (http://www.icd9data.com/getICD9Code.ashx?icd9=170.9)
ICD-O:
M9260/3 (http://www.progenetix.net/progenetix/I92603/)
OMIM
133450 (http://omim.org/entry/133450)
DiseasesDB
4604 (http://www.diseasesdatabase.com/ddb4604.htm)
MedlinePlus
001302(http://www.nlm.nih.gov/medlineplus/ency/article/001302.htm)
eMedicine
ped/2589 (http://www.emedicine.com/ped/topic2589.htm)
MeSH
D012512 (http://www.nlm.nih.gov/cgi/mesh/2011/MB_cgi?field=uid&term=D012512)
Ewing sarcoma
is amalignant round-cell tumour.It is a rare disease in whichcancer cells are found in thebone or in soft tissue. Themost common areas in whichit occurs are the pelvis, thefemur, the humerus, the ribsand clavicle.Because a common geneticlocus is responsible for a largepercentage of Ewing sarcomaand primitive neuroectodermaltumors, these are sometimesgrouped together in a categoryknown as the Ewing family of tumors.
[1]
The diseases are,however, considered to bedifferent: peripheral primitiveneuroectodermal tumours aregenerally not associated withbones, while Ewing sarcomasare most commonly related tobone.Ewing sarcoma occurs mostfrequently in male teenagers,with a male/female ratio of 1.6:1.
[2]
Although usually classified as a bone tumour, Ewing sarcoma can have characteristics of bothmesodermal and ectodermal origin, making it difficult to classify.
[3]
Contents
1 Eponym2 Causes3 Clinical findings4 Imaging findings5 Clinical differential diagnosis6 Diagnosis7 Epidemiology8 Treatment8.1 Fertility preservationPage 1 of 4Ewing's sarcoma - Wikipedia, the free encyclopedia7/7/2011http://en.wikipedia.org/wiki/Ewing's_sarcoma
 
 
Eponym
James Ewing (18661943) first described the tumour, establishing that the disease was separate fromlymphoma and other types of cancer known at that time.
[4][5]
Causes
Genetic exchange between chromosomes can cause cells to become cancerous. Ewing sarcoma is theresult of a translocation between chromosomes 11 and 22, which fuses the EWS gene of chromosome 22to 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 apeak between 10 and 20 years of age. It can occur anywhere in the body, but most commonly in thepelvis and proximal long tubular bones, especially around the growth plates. The diaphyses of the femurare the most common sites, followed by the tibia and the humerus. Thirty percent are overtly metastaticat presentation. Patients usually experience extreme bone pain.It is positive for CD99 and negative for CD45.
[9]
Imaging findings
On conventional radiographs, the most common osseous presentation is apermeative lytic lesion with periosteal reaction. The classic description of lamellated or "onion skin" type periosteal reaction is often associated with thislesion. Plain films add valuable information in the initial evaluation or screening.The wide zone of transition (e.g. permeative) is the most useful plain filmcharacteristic in differentiation of benign versus aggressive or malignant lyticlesions.MRI should be routinely used in the work-up of malignant tumours. MRI will showthe full bony and soft tissue extent and relate the tumour to other nearby anatomicstructures (e.g. vessels). Gadolinium contrast is not necessary as it does not giveadditional information over noncontrast studies, though some current researchersargue that dynamic, contrast enhanced MRI may help determine the amount of necrosis within the tumour, thus help in determining response to treatment prior tosurgery.Page 2 of 4Ewing's sarcoma - Wikipedia, the free encyclopedia7/7/2011http://en.wikipedia.org/wiki/Ewing's_sarcoma
 
X-Ray of a childwith Ewingsarcoma of thetibia.
 
Magnetic resonance imaging sliceshowing Ewing's sarcoma of the lefthip (white area shown right).
 
Micrograph of a metastatic Ewingsarcoma with the characteristiccytoplasmic clearing on H&Estaining, which was showing to bePAS positive.
CT can also be used to define the extraosseous extent of the tumour, especially inthe skull, spine, ribs and pelvis. Both CT and MRI can be used to follow responseto radiation and/or chemotherapy.Bone scintigraphy can also be used to follow tumour response to therapy.In the group of malignant small round cell tumours whichinclude Ewing's sarcoma, bone lymphoma and small cellosteosarcoma, the cortex may appear almost normalradiographically, while there is permeative growth throughoutthe Haversian channels. These tumours may be accompanied bya large soft tissue mass while there is almost no visible bonedestruction. The radiographs frequently do not shown any signsof cortical destruction.
Clinical differential diagnosis
Other entities that may have a similar clinical presentationinclude osteomyelitis, osteosarcoma (especially telangiectaticosteosarcoma) and eosinophilic granuloma. Soft tissueneoplasms such as pleomorphic undifferentiated sarcoma(malignant fibrous histiocytoma) that erode into adjacent bonemay also have a similar appearance.
Diagnosis
The definitive diagnosis is based on histomorphologic findings,immunohistochemistry and molecular pathology.Ewing sarcoma is a small round cell tumor, that typically has aclear cytoplasm on H&E staining, due to glycogen. The presenceof the glycogen can be demonstrated with positive PAS stainingand negative PAS diastase staining. The characteristicimmunostain is CD99 which diffusely marks the cell membrane.Morphologic and immunohistochemical findings arecorroborated with an associated chromosomal translocation, of which there are several. The most common translocation, presentin approximately 90% of Ewing sarcoma cases, is t(11;22)(q24;q12).
[10][11]
The pathologic differential diagnosis is the grouping of smallround cell tumors, which includes lymphoma, alveolarrhabdomyosarcoma and desmoplastic small round cell tumor, among others.
Epidemiology
The frequency in the United States depends on the patient's age, with a rate of 0.3 case per 1,000,000children in those younger than 3 years of age to as high as 4.6 cases per 1,000,000 in adolescents aged1519 years. Internationally the annual incidence rate averages less than 2 cases per 1,000,000 children.Page 3 of 4Ewing's sarcoma - Wikipedia, the free encyclopedia7/7/2011http://en.wikipedia.org/wiki/Ewing's_sarcoma

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