Ewing sarcoma


Distribution of Ewing's sarcoma: Most frequent locations are the large long bones and the pelvis.
  • Highly aggressive malignant bone or soft tissue tumor arising from cells of the neuroectoderm
  • One-fourth of patients will have metastasis at the time of presentation
    • It is assumed that almost all patients have subclinical metastasis
  • Presence and location of metastasis is the major prognostic factor for survival
  • Metastasis is most frequently seen in bone (spine most common) and lungs
  • Around 70 to 80 percent survival in those with non-metastatic disease
  • More common in males

Clinical Features

  • Constant pain for months over site of malignancy
    • Worse with exercise and at night
  • Site will be edematous and tender to palpation
    • Mass can sometimes be appreciated
  • Most often found in pelvis, axial skeleton, or diaphysis of femur with a small minority arising in soft tissues
  • Pelvic tumors are more regularly associated with metastatic disease
  • Fever, weight loss, fatigue, night sweats occur in less than 20 percent of patients
  • Pathological fractures can occur

Differential Diagnosis

Bone tumors and their mimics





X-ray of a child with Ewing's sarcoma of the tibia
MRI showing Ewing's sarcoma of the left hip (white area)
  • Appearance on plain radiographs often described as “onion peel” or “moth-eaten” appearance referring to the respective periosteal bone formation and osseous destruction
  • LDH is a prognostic factor
  • Definitive diagnosis is made with biopsy
  • Work-up often includes PET scan for metastatic disease
  • t(11;22) translocation is often seen


  • Pain control
  • Neoadjuvant and adjuvant chemotherapy
  • Surgery and/or radiation therapy for local control of disease


  • Home with outpatient follow up
  • Admission if necessary for pain control or significant disease burden


  1. Niederhuber, John E., et al. “Sarcomas.” Abeloff's Clinical Oncology E-Book. Elsevier Health Sciences, 2019, pp. 1604 – 1654.e8.