Treatment of Ewing's Sarcoma of the Chest
Ewing's sarcoma of the chest requires multimodal treatment consisting of combination chemotherapy (3-6 cycles induction, then 6-10 cycles consolidation), followed by local control with surgery as the preferred modality, or radiotherapy when surgery is not feasible, with total treatment duration of 8-12 months. 1
Treatment Algorithm
Initial Management
- All patients must be referred immediately to a specialized sarcoma center before any biopsy or treatment initiation, as this is the accepted standard for these rare and complex tumors 1, 2
- Chest CT scan is mandatory to rule out lung or pleural metastases before treatment planning 1
- Complete staging includes bone scintigraphy and bone marrow aspirates from sites distant to the primary tumor 1
Chemotherapy Regimen
- The most effective protocols include at least one alkylating agent (ifosfamide or cyclophosphamide) combined with doxorubicin 1
- Active agents include: doxorubicin, cyclophosphamide, ifosfamide, vincristine, dactinomycin, and etoposide 1, 3
- Treatment consists of 3-6 cycles of induction chemotherapy after biopsy, followed by local therapy, then 6-10 additional consolidation cycles at 3-week intervals 1
- The incorporation of ifosfamide and etoposide significantly improved outcomes in randomized trials 1
- Total of 12-15 cycles over 8-12 months duration 3
Local Control Strategy
For Chest Wall Tumors:
- Complete surgical resection with wide margins is the preferred local control modality when feasible 1
- Surgery alone achieved 0% local recurrence rate at 5 years in appropriately selected patients 4
- Radiotherapy should be applied if complete surgery is impossible 1
- Radiotherapy should also be considered when histological response to preoperative chemotherapy was poor (>10% viable tumor cells in the surgical specimen) 1
Radiotherapy Dosing:
- 40-45 Gy for microscopic residual disease 1, 3
- 50-60 Gy for macroscopic disease 1, 3
- Local control after radiation alone or combined surgery plus radiation was 81% at 5 years in chest wall tumors 4
Expected Outcomes
- With multimodal therapy, 5-year survival approximates 60-70% in localized disease 1
- Surgery or radiotherapy alone yields <10% 5-year survival 1
- Overall survival for chest wall Ewing's sarcoma is 60% at 5 years with event-free survival of 50% 4
Prognostic Factors Specific to Chest Wall Location
- Axial localization (including chest wall) is an adverse prognostic factor 1
- Other adverse factors include: tumor size >8-10 cm, age >15 years, elevated LDH, and poor histological response to chemotherapy 1, 3, 2
- Incomplete or no surgery for local therapy represents an additional adverse prognostic factor 1
Critical Pitfalls to Avoid
- Incomplete surgery followed by radiotherapy was not superior to radiotherapy alone in large series, so if complete resection cannot be achieved, radiotherapy alone should be strongly considered 1
- Delayed referral to specialized centers can contaminate tissue planes and compromise subsequent surgical management 2
- Central radiotherapy planning significantly improved local control compared to non-standardized approaches 4
- In chest wall tumors, marginal relapses (outside radiation fields but near them) occurred in 4 of 10 evaluable local failures, emphasizing the importance of adequate radiation field design 4
Metastatic Disease Considerations
- Approximately 10% of patients present with lung metastases at diagnosis 1
- Lung/pleural metastases have better prognosis than bone metastases (20-40% versus <20% 5-year survival) 1
- Resection of residual lung metastases after chemotherapy and whole lung irradiation may confer survival advantage 1
- Patients with metastatic disease should receive therapy similar to localized disease with appropriate local treatment of metastases 1