Ewing

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Ewings 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 gt Females
• 65 in the 2nd 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
• Reciprocal translocation

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Pathology

• Gross soft, tan, gray tissue
• Micro nests of small round cells
• No osteoid or chondroid production
• Surface cell glycoprotein

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Pathology

• Differential Diagnosis
• Lymphoma
• Osteomyelitis
• OGS
• EG
• MFH
• Metastatic neuroblastoma

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Staging and Prognosis

• Local and distal staging
• Bone marrow aspirate
• Pre-chemotherapy investigations
• Biopsy

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Staging and Prognosis

• Location
• Tumor Size
• Metastases at diagnosis
• Response to chemotherapy

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Treatment

• Multidisciplinary approach
• Neoadjuvant chemotherapy, surgery
• Dont prolong interval between chemo
• Radiation if indicated

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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