From the Guidelines
The 5-year survival rate for Ewing's sarcoma with lung metastases is approximately 30%, which is better than skeletal metastases with a 10% 5-year survival rate. Patients with isolated lung metastases tend to have better outcomes than those with bone or bone marrow metastases 1. Treatment typically involves intensive multimodal therapy including systemic chemotherapy (usually with vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide), surgical resection of lung nodules when possible, and radiation therapy.
Key Factors Influencing Survival
- The number and size of lung metastases
- Response to initial chemotherapy
- Age of the patient
- Whether the lung metastases were present at diagnosis or developed later as recurrence Younger patients and those with fewer, smaller metastatic nodules that respond well to initial chemotherapy generally have better outcomes. Complete surgical removal of all visible lung metastases, when feasible, can significantly improve survival chances.
Treatment Approach
Patients with metastatic disease at diagnosis should have the same standardized chemotherapy as patients with localized disease 1. For patients with lung metastases who achieve a complete remission, total lung irradiation should be considered, and thoracotomy should be evaluated for patients with limited residual macroscopic disease. Supplemental irradiation of bone metastases is usually indicated.
Importance of Specialized Care
Because Ewing’s sarcoma is a rare disease with a complex management, patients should be treated in specialized centers 1. This ensures that patients receive the most appropriate and effective treatment, which can significantly impact their survival and quality of life.
From the Research
Ewing's Sarcoma Lung Mets Survival
- The survival rate for Ewing's sarcoma patients with lung metastases is generally poor, with a 2-year overall survival rate of 70.6% 2.
- A study found that the median event-free survival and overall survival for patients with extensive lung/pleural metastases who achieved a complete response to systemic therapy were 9.3 months and 37.5 months, respectively 2.
- The presence of metastases at diagnosis is a predictive factor for poor prognosis, with a 3-year disease-free survival rate of 21% for patients with metastases compared to 70% for those without metastases 3.
- Local control can be achieved in the majority of patients using radiotherapy exclusively, even among those with bulky, central axis tumors 4.
- However, recurrence exactly in situ is a common relapse pattern, and whole lung irradiation may not be sufficient to prevent local recurrence in lung or pleura 2.
- Emerging therapies, such as integrating novel molecular targets with conventional chemotherapeutic agents, may hold promise for high-risk Ewing's sarcoma patients with distant metastasis 5.
- The 5-year disease-free and overall survival rates for Ewing's sarcoma patients are around 50-59% and 56-59%, respectively, with no significant advantage observed when ifosfamide and etoposide are added to the VACA regimen 6.