From the Guidelines
Ewing's sarcoma of the leg is significantly more treatable now than in the 1980s, with modern treatment approaches prioritizing chemotherapy regimens like VDC/IE, which has demonstrated superiority over VIDE, as recommended in the 2025 UK guidelines for the management of bone sarcomas 1. The treatment of Ewing's sarcoma has evolved substantially since the 1980s, with a shift towards a multimodal approach that combines chemotherapy, surgery, and radiation therapy.
- Key aspects of modern treatment include:
- Systemic treatment with chemotherapy as the standard, with the VDC/IE regime being preferred over VIDE due to its demonstrated superiority 1.
- Local treatment decisions that are complex and require thorough multidisciplinary team (MDT) discussion, including presentation at national multidisciplinary team meetings for Ewing sarcoma 1.
- The use of surgery, radiotherapy, or both to treat all structures involved in the pre-chemotherapy volume when treating the primary tumor with curative intent 1.
- Consideration for clinical trials for patients with newly diagnosed and relapsed disease, reflecting the ongoing effort to improve outcomes 1. Given the advancements in treatment protocols and supportive care, the current approach to Ewing's sarcoma of the leg offers improved outcomes in terms of morbidity, mortality, and quality of life compared to the 1980s.
- Improvements in imaging technologies, molecular diagnostics, and surgical techniques have all contributed to better patient outcomes.
- The emphasis on limb-sparing procedures and more precise radiation therapy has significantly reduced the need for amputations, improving the quality of life for patients with leg tumors. Overall, while challenges remain, particularly with metastatic disease, the modern treatment of Ewing's sarcoma of the leg is more effective and offers better patient outcomes than the treatments available in the 1980s, as supported by the latest guidelines and research 1.
From the Research
Treatment Options for Ewing Sarcoma
- The treatment for Ewing sarcoma involves a combination of chemotherapy, surgery, and radiation therapy, with the goal of achieving a cure for the majority of patients 2, 3.
- The standard treatment approach includes 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 choice of local treatment, such as surgery or radiation therapy, depends on various factors, including patient characteristics, tumor location, and response to neoadjuvant chemotherapy, and should be decided by a multidisciplinary tumor board together with the patient 4.
Comparison of Treatment Outcomes
- Studies have shown that the addition of ifosfamide and etoposide to the conventional VACA regimen did not improve treatment outcomes for patients with Ewing sarcoma 5.
- The 5-year disease-free and overall survival rates for patients with Ewing sarcoma have improved over time, with current rates ranging from 54% to 59% 5, 6.
- International collaborations have defined the current standard of care for Ewing sarcoma, delivering multiple cycles of systemic therapy combined with local treatment, which has achieved a realistic chance of cure for the majority of patients 3.
Advances in Treatment
- Recent studies have focused on optimizing treatment strategies for patients with Ewing sarcoma, including the use of interval compressed chemotherapy and high-dose chemotherapy with autologous stem cell transplantation 6.
- The development of consensus statements for the upfront management of patients with Ewing sarcoma has provided clarity and recommendations for treating physicians 6.
- Ongoing research aims to overcome the hurdles of treatment-associated morbidities and improve treatment outcomes for patients with Ewing sarcoma 3.