From the Guidelines
The prognosis for a patient with advanced Ewing's Sarcoma with pulmonary metastases is generally poor, with a 5-year survival rate of around 20-30%, although individual outcomes can vary significantly based on several factors, including response to initial chemotherapy and ability to achieve complete surgical removal of all visible disease.
Factors Influencing Prognosis
- The patient's age, with younger patients often faring better 1
- Overall health status
- Tumor burden
- Response to initial chemotherapy, with early response being a strong predictor of outcome 1
- Whether complete surgical removal of all visible disease is possible
Treatment Approach
Treatment typically involves a multimodal approach including:
- Systemic chemotherapy, usually with vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide 1
- Surgery when possible
- Radiation therapy, with whole lung irradiation indicated for patients with pulmonary metastases who achieve a complete remission 1
Recent Guidelines
The most recent guidelines, as outlined in the 2025 UK guidelines for the management of bone sarcomas 1, recommend a standardized chemotherapy approach for patients with metastatic disease, similar to that for localized disease, with consideration of whole lung irradiation for patients with pulmonary metastases.
Key Considerations
- The role of high-dose therapy with autologous stem cell rescue is not established, and standard systemic therapy for high-risk disseminated disease remains interval-compressed VDC/IE chemotherapy 1
- Radiotherapy for bone metastases can provide palliation and local control, although toxicity can be significant 1
- Surgical resection of residual metastases is less well-defined, but may be considered in select cases 1
From the Research
Prognosis for Advanced Ewing's Sarcoma with Pulmonary Metastases
The prognosis for patients with advanced Ewing's Sarcoma and pulmonary metastases remains poor, even with complete response to systemic therapy 2.
Survival Outcomes
- The median event-free survival (EFS) and overall survival (OS) for patients with extensive lung/pleural metastases were 9.3 months and 37.5 months, respectively 2.
- The 2-year EFS was 19% and the 2-year OS was 70.6% 2.
- Patients who underwent surgical resection of lung metastases had a better overall survival (80%) compared to those who received radiation alone or chemotherapy only 3.
- The 5-year overall survival rate for patients with metastatic Ewing sarcoma was 22.1% 4.
Treatment Options
- Whole-lung irradiation (WLI) is not sufficient to prevent local recurrence in lung or pleura, and more aggressive local treatment for metastatic lesions is warranted 2.
- Surgical resection of lung metastases may have a role in the treatment of highly selected patients with Ewing's Sarcoma and isolated lung metastases, depending on the histologic response to therapy 5.
- Whole-lung radiotherapy (WLRT) may improve survival outcomes in patients with metastatic Ewing sarcoma limited to pulmonary metastases, with trends of improved progression-free survival (PFS) and overall survival (OS) at five years 6.
- Aggressive primary treatment, including radiotherapy to metastatic sites, should be considered in patients with uncommon sites of metastasis 4.
Recurrence Patterns
- Lung/pleural relapse occurred in 71.4% of patients with complete response or near-complete response to systemic therapy, with most recurrent lesions exactly coinciding with the original metastatic lesions before chemotherapy 2.
- The major relapse pattern was recurrence exactly in situ, with 42.9% of patients having distant metastases other than lung/pleural metastases 2.