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EWING'S SARCOMA

• Ewing’s sarcoma is a small round-cell tumor typically arising in the


bones, rarely in soft tissues, of children and adolescents.
• All are characterised by recurrent chromosomal translocation
involving 11 & 22 (85%) & 21 &22 (15%)
Epidemiology

2nd most common primary osseous


malignancy in children
Incidence is 2.1 / million
Rare in African-Americans and Asians
80% occur between ages 5 and 25

Male : Female
1.3:1 < 10 yrs
1.6:1 > 10 yrs
CLINICAL
MANIFESTATION
almost always metaphyseal or
diaphyseal
• Localised, painful, tender mass
• Systemic symp. – fever, malaise,
weight loss
• mistaken for OM
• dissemination of tumor
• May metastasize to otherbones
• Elevated WBC & ESR,LDH
• pathological fracture
RADIOGRAPH
• The lesion is poorly defined
• Permeative or moth-eaten
type of bone destruction
• Aggressive periosteal
response
onion peel
codman triangle
sunburst appearance

• Large soft-tissue mass

• Occasionally, the bone lesion


itself is almost imperceptible,
with the soft-tissue massbeing
the only prominent feature
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HISTOPATHOLOGY
HIGHPOWER

• Cells are uniform and round


to oval

• Cytoplasm is scanty & lacy

• Nuclei are round to oval,


have a delicate ,finely
dispersed chromatin

• Nucleoli are inconspicuous

• Mitotic figures rare


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TREATMENT
MULTIDISCIPLINARYAPPROACH

1. CHEMOTHERAPY- control ofmicrometastasis

2. SURGERY - local control where possible

3. RADIOTHERAPY-local control where surgery


is not possible or incomplete
Radiosensitive tumor
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Prognosis
• Poor prognostic factors:
• Age >17ys
• Large size tumor > 8cm
• Metastases
• Pelvic & Sacraltumors
• Fever, Leucocytosis & Raised ESRat presentation
• <90% necrosis after Chemo
• Extraosseous soft tissue extension
• Postradiation medullary tumor
• Small bones(strong CD99+ve) – better prognosis

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