Ewing’s Sarcoma

Ewing’s Sarcoma - Reciprocal translocation...

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Ewing’s Sarcoma Rob Masnyk MD May 10, 2001
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Overview Introduction Epidemiology Clinical Presentation Radiology Pathology Staging, Prognosis, Treatment
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Introduction Identified in 1921 by James Ewing Differed from osteogenic sarcoma Different location Poor survival: 5-10% at 5 years
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Introduction 1967: radiation primarily 1969: adjuvant chemotherapy More long term complications
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Epidemiology Incidence is 0.6 per million Males > Females 65% in the 2 nd decade of life Rare in blacks and Asians
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Clinical Presentation History: Pain most commonly (90%) Swelling (70%) Fever (20%) Pathological fracture Weight loss, malaise
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Clinical Presentation Physical Exam and Labs Local warmth, inflammation Pleural effusions Neurological signs if spinal involvement ESR, LDH, anemia, leukocytosis
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Radiology Site Size Effect on bone Response of Bone Matrix Cortex Soft tissue
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Pathology Cell of origin is unknown Previously a diagnosis of exclusion
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Unformatted text preview: Reciprocal translocation Pathology Gross: soft, tan, gray tissue Micro: nests of small round cells No osteoid or chondroid production Surface cell glycoprotein Pathology Differential Diagnosis Lymphoma Osteomyelitis OGS EG MFH Metastatic neuroblastoma Staging and Prognosis Local and distal staging Bone marrow aspirate Pre-chemotherapy investigations Biopsy Staging and Prognosis Location Tumor Size Metastases at diagnosis Response to chemotherapy Treatment Multidisciplinary approach Neoadjuvant chemotherapy, surgery Dont prolong interval between chemo Radiation if indicated Summary Rare but common Main ddx is lymphoma and infection Large soft tisse masses Neoadjuvant chemo and surgery 75-80% disease free survival at 5 years...
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Ewing’s Sarcoma - Reciprocal translocation...

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