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