From the Guidelines
The primary treatment approach for Ewing sarcoma should involve a combination of chemotherapy, surgery, and radiation therapy, with surgery being the preferred method for local control, as evidenced by the ESMO clinical recommendations 1. When it comes to treating Ewing sarcoma, a multidisciplinary approach is essential. This includes:
- Chemotherapy: The standard regimen typically consists of vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide (VDC/IE) 1.
- Surgery: The goal is to remove the tumor completely when possible, while preserving function. A wide surgical margin should be attempted 1.
- Radiation therapy: This may be used when complete surgical removal isn't feasible or to address microscopic disease, with radiation doses depending on the tumor site, ranging from 40-45 Gy for microscopic residual disease to 50-60 Gy for macroscopic disease 1.
In terms of follow-up, patients should be monitored at regular intervals, including:
- 3-month intervals until 3 years after the end of treatment
- 6-month intervals until 5 years
- 8-12 month intervals until at least 10 years 1 It's crucial to note that patients with Ewing sarcoma are at risk of late relapse, long-term toxicity, and secondary cancers, emphasizing the importance of prolonged follow-up 1. Overall, the treatment strategy should prioritize minimizing morbidity, mortality, and optimizing quality of life, with a focus on early diagnosis and a multidisciplinary approach 1.
From the Research
Overview of Ewing Sarcoma
- Ewing sarcoma is a rare tumor that requires complex multidisciplinary management 2
- It is a highly aggressive bone and soft-tissue cancer with a dismal prognosis for patients with macrometastatic or recurrent disease 3
Treatment Approaches
- Standard treatment involves multiagent induction chemotherapy followed by local treatment with surgery, definitive radiation, or a combination of surgery and radiation followed by additional chemotherapy and consolidation local treatment to metastatic sites 2
- The combination of non-directed chemotherapeutics and ever-evolving local modalities achieves a realistic chance of cure for the majority of patients with Ewing sarcoma 3
- Alternating vincristine, doxorubicin, cyclophosphamide, and ifosfamide, etoposide (VDC/IE)-based multimodality therapy has shown favorable outcomes in adults with localized Ewing sarcoma 4
Local and Metastasis-Directed Therapies
- Local/metastasis directed therapy (LMDT) should be considered on a case-by-case basis to assess appropriateness, optimal timing/modality, palliative versus curative intent, and its role in relation to chemotherapy in the management of relapsed/recurrent Ewing sarcoma (RR-ES) 5
- Radiotherapy (RT) is a commonly used LMDT, which can be delivered through standard, hypofractionated, or stereotactic techniques based on factors including prior RT, tumor size, and/or location 5
- Surgery for LMDT not only addresses the tumor but also provides tissue for analysis, though the potential surgical morbidity based on location, extent of resection, and recovery complications should be considered 5
Prognosis and Outcomes
- Actuarial 5-year event-free survival (EFS) and overall survival rates were 42% and 45%, respectively, with a median duration of potential follow-up of 6.8 years in one study 6
- Five-year local recurrence-free survival (LRFS) was 91%, and 5-year overall survival (OS) was 79% in adults with localized Ewing sarcoma treated with VDC/IE-based multimodality therapy 4
- Pelvic site of disease remains a predictor of worse outcome 4