Ewing’s sarcoma
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Transcript of Ewing’s sarcoma
Ewing’s Sarcoma
Overview
• Introduction
• Epidemiology
• Clinical Presentation
• Radiology
• Pathology
• Staging, Prognosis, Treatment
Introduction
• Identified in 1921 by James Ewing
• Differed from osteogenic sarcoma
• Different location
• Poor survival: 5-10% at 5 years
Introduction
• 1967: radiation primarily
• 1969: adjuvant chemotherapy
• More long term complications
Epidemiology
• Incidence is 0.6 per million
• Males > Females
• 65% in the 2nd decade of life
• Rare in blacks and Asians
Clinical Presentation
History:
• Pain most commonly (90%)
• Swelling (70%)
• Fever (20%)
• Pathological fracture
• Weight loss, malaise
Clinical Presentation
Physical Exam and Labs• Local warmth, inflammation
• Pleural effusions
• Neurological signs if spinal involvement
• ESR, LDH, anemia, leukocytosis
Radiology
• Site
• Size
• Effect on bone
• Response of Bone
• Matrix
• Cortex
• Soft tissue
Pathology
• Cell of origin is unknown
• Previously a diagnosis of exclusion
• 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
• Don’t 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