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| {{Infobox_Disease |
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| Name = Ewing's sarcoma |
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| Image = EwingSarcomaTibia.jpg |
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| Caption = X-Ray of a child with Ewing's sarcoma of the tibia |
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| DiseasesDB = 4604 |
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| ICD10 = |
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| ICD9 = {{ICD9|170.9}} |
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| ICDO = 9260/3 |
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| OMIM = |
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| MedlinePlus = 001302 |
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| }}
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| {{Ewing's sarcoma}} | | {{Ewing's sarcoma}} |
| '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''.
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| {{CMG}}; '''Assistant Editor(s)-In-Chief:''' [[User:Michael Maddaleni|Michael Maddaleni, B.S.]]
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| ==Overview==
| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
| '''Ewing's sarcoma''' is the common name for ''[[primitive neuroectodermal tumor]]''. It is a rare disease in which [[cancer]] cells are found in the [[bone]] or in soft [[biological tissue|tissue]]. The most common areas in which it occurs are the [[pelvis]], the [[femur]], the [[humerus]], and the [[ribs]]. [[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. Ewing's sarcoma occurs most frequently in male teenagers. | |
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| Ewing's 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.
| | {{CMG}};{{AE}} {{PSK}} '''Assistant Editor(s)-In-Chief:''' [[User:Michael Maddaleni|Michael Maddaleni, B.S.]] |
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| ==Epidemiology==
| | {{SK}} Ewing sarcoma; Ewings sarcoma |
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| 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. In the United Kingdom an average of six children per year are diagnosed, mainly males in early stages of puberty.
| | ==[[Ewing's sarcoma overview|Overview]]== |
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| 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.
| | ==[[Ewing's sarcoma historical perspective|Historical Perspective]]== |
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| ==Clinical findings== | | ==[[Ewing's sarcoma classification|Classification]]== |
| Ewing's 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 [[metastasis|metastatic]] at presentation. | |
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| The most common clinical findings are pain and swelling diarrhea.
| | ==[[Ewing's sarcoma pathophysiology|Pathophysiology]]== |
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| ==Imaging findings== | | ==[[Ewing's sarcoma causes|Causes]]== |
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| On conventional [[radiography|radiographs]], the most common osseous presentation is a permeative lytic lesion with [[periosteum|periosteal]] reaction. The classic description of lamellated or "onion skin" type [[periosteum|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 differention of benign versus aggressive or malignant lytic lesions.
| | ==[[Differentiating Ewing's sarcoma from other diseases|Differentiating Ewing's sarcoma from other diseases]]== |
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| [[Magnetic resonance imaging|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 non contrast 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. | | ==[[Ewing's sarcoma epidemiology and demographics|Epidemiology and Demographics]]== |
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| [[Computed axial tomography|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]]. | | ==[[Ewing's sarcoma risk factors|Risk Factors]]== |
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| Bone [[scintigraphy]] can also be used to follow tumor response to therapy.
| | ==[[Ewing's sarcoma screening|Screening]]== |
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| ==Differential diagnosis== | | ==[[Ewing's sarcoma natural history|Natural History, Complications and Prognosis]]== |
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| Other entities that may have a similar radiologic 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. | | ==Diagnosis== |
| | [[Ewing's sarcoma staging|Staging]] | [[Ewing's sarcoma history and symptoms|History and Symptoms]] | [[Ewing's sarcoma physical examination|Physical Examination]] | [[Ewing's sarcoma laboratory tests|Laboratory Findings]] | [[Ewing's sarcoma x ray|X Ray]] | [[Ewing's sarcoma CT|CT]] | [[Ewing's sarcoma MRI|MRI]] | [[Ewing's sarcoma other imaging findings|Other Imaging Findings]] | [[Ewing's sarcoma other diagnostic studies|Other Diagnostic Studies]] |
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| ==Treatment== | | ==Treatment== |
| | [[Ewing's sarcoma medical therapy|Medical therapy]] | [[Ewing's sarcoma surgery|Surgery]] | [[Ewing's sarcoma primary prevention|Primary prevention]] | [[Ewing's sarcoma secondary prevention|Secondary prevention]] | [[Ewing's sarcoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Ewing's sarcoma future or investigational therapies|Future or Investigational Therapies]] |
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| Because almost all patients with apparently localized disease at diagnosis have occult metastatic disease, multi drug [[chemotherapy]] as well as local disease control with surgery and/or radiation is indicated in the treatment of all patients. 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.
| | ==Case Studies== |
| | [[Ewing's sarcoma case study one|Case#1]] |
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| ==Prognosis== | | ==External Links== |
| | * [http://cancer.gov/cancertopics/pdq/treatment/ewings/healthprofessional National Cancer Institute listing for Ewing's sarcoma] |
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| Staging attempts to distinguish patients with localized from those with [[metastasis|metastatic]] disease. Most commonly, [[metastasis|metastases]] occur in the chest, bone and/or bone marrow. Less common sites include the [[central nervous system]] and [[lymph node]]s.
| | [[fr:Sarcome d'Ewing]] |
| | | [[pl:Mięsak Ewinga]] |
| Survival for localized disease is 65-70% when treated with [[chemotherapy]]. Long term survival for [[metastasis|metastatic]] disease can be less than 10% but some sources state it is 25-30%.
| | [[pt:Sarcoma de Ewing]] |
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| ==References==
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| {{reflist|2}}
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| ==External links==
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| * [http://cancer.gov/cancertopics/pdq/treatment/ewings/healthprofessional National Cancer Institute listing for Ewing's sarcoma]
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| [[Category:Disease state]] | | [[Category:Disease]] |
| [[Category:Orthopedics]] | | [[Category:Orthopedics]] |
| [[Category:Types of cancer]] | | [[Category:Types of cancer]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |
| [[Category:Mature chapter]] | | [[Category:Mature chapter]] |
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| [[de:Ewing-Sarkom]]
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| [[fr:Sarcome d'Ewing]]
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| [[nl:Ewing-sarcoom]]
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| [[pl:Mięsak Ewinga]]
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| [[pt:Sarcoma de Ewing]]
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| [[fi:Ewingin sarkooma]]
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| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
| {{WikiDoc Sources}} | | {{WikiDoc Sources}} |
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| | [[Category:Oncology]] |
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| | [[Category:Orthopedics]] |