What is the standard treatment for Ewing's sarcoma?

Standard Treatment for Ewing's Sarcoma

The standard treatment for Ewing's sarcoma consists of a multidisciplinary approach combining intensive chemotherapy, local control with surgery when possible, and radiation therapy for inoperable tumors or inadequate surgical margins, which has significantly increased 5-year survival rates from less than 10% to approximately 60%. 1

Diagnostic Approach

• Patients with radiological findings suggesting bone sarcoma should be referred without prior biopsy to a specialized bone sarcoma center 1
• Diagnosis is made by needle biopsy or open surgical biopsy, with detection of characteristic translocations (most commonly t(11;22)(q24;q12)) by cytogenetics or PCR being diagnostic 1
• Before treatment, comprehensive staging should include:
  • Radiological evaluation of the entire affected bone 1
  • CT scan for pulmonary metastases 1
  • Bone scintigraphy to investigate bone metastases 1
  • Bone marrow aspirates for microscopic examination 1

Treatment Algorithm

1. Chemotherapy

• Induction Phase:
  • 3-6 courses of combination chemotherapy 1, 2
  • Standard drugs include doxorubicin, vincristine, ifosfamide, etoposide, dactinomycin, and cyclophosphamide 1, 2

2. Local Control (after induction chemotherapy)

• Surgery: Preferred treatment for local control when possible 1, 2

  • Wide surgical margins should be attempted 1

• Radiation Therapy: Used in the following scenarios:

  • Inoperable tumors 1, 2
  • Marginal or intralesional surgical margins 1, 2
  • Radiation doses:
    • 40-45 Gy for microscopic residual disease 1, 2
    • 50-60 Gy for macroscopic disease 1, 2
  • Hyperfractionated regimens may be considered for optimal integration with chemotherapy 1

3. Consolidation Chemotherapy

• 8-10 additional courses of chemotherapy 1, 2
• Total treatment duration: 8-12 months 1
• Total number of chemotherapy courses: 12-15 1

Management of Metastatic Disease

• Patients with metastatic disease should receive the same standardized chemotherapy as those with localized disease 1
• Additional interventions for specific metastatic sites:
  • Lung metastases: Total lung irradiation should be considered for patients achieving complete remission; thoracotomy for those with limited residual disease 1
  • Bone metastases: Supplemental irradiation is usually indicated 1

Prognostic Factors

• Adverse prognostic factors include:
  • Metastatic disease at diagnosis 1
  • Pelvic localization 1
  • Tumor diameter >8-10 cm 1
  • Age >15 years 1
  • Elevated serum LDH 1
  • Poor histological response to preoperative chemotherapy 1
  • Radiotherapy as the only local treatment 1

Follow-up Recommendations

• Every 3 months until 3 years after treatment completion 1
• Every 6 months until 5 years 1
• Every 8-12 months until at least 10 years 1
• Extended follow-up is recommended due to risk of late relapse and long-term toxicity 1

Important Considerations and Pitfalls

• Approximately 20% of patients have detectable metastases at diagnosis 1
• Prognosis varies significantly based on metastatic status:
  • Localized disease: 65-75% 5-year survival 3
  • Isolated pulmonary metastases: ~50% 5-year survival 3
  • Other metastatic disease: <30% 5-year survival 3
• Sperm banking should be considered before treatment initiation due to potential fertility impacts 1
• Patients with recurrent disease generally have poor prognosis and should be considered for palliative care, except those with limited relapse after a long disease-free interval 1