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 stated in the ESMO clinical recommendations 1. When it comes to treating Ewing sarcoma, the goal is to achieve the best possible outcome in terms of morbidity, mortality, and quality of life.
- The treatment plan typically includes chemotherapy, with the standard regimen consisting of vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide (VDC/IE), administered over several cycles.
- Surgery aims to remove the tumor completely when possible, with a wide surgical margin attempted to minimize the risk of recurrence.
- Radiation therapy may be used before or after surgery to target remaining cancer cells, with the radiation dose depending on the tumor site and extent of disease. Some key considerations in the treatment of Ewing sarcoma include:
- For patients with metastatic disease at diagnosis, the same standardized chemotherapy as patients with localized disease should be used 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 1.
- Patients should be followed at regular intervals after treatment to monitor for recurrence and potential long-term toxicity 1. The follow-up care should be tailored to the individual patient's needs, with regular assessments and interventions as necessary to optimize outcomes and minimize complications 1.
From the Research
Treatment Options for Ewing Sarcoma
- Surgery alone or radiation therapy (RT) alone are two reasonable treatment options for patients with localized Ewing's sarcoma of bone following neoadjuvant chemotherapy 2
- The combination of surgery plus RT is not recommended as an initial treatment option 2
- The local treatment for an individual patient should be decided by a multidisciplinary tumour board together with the patient after consideration of patient characteristics, potential benefits and complications, and patient preferences 2
Role of Chemotherapy and Radiation
- 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 3
- The integration of ifosfamide and etoposide into the front-line therapy of patients with Ewing's sarcoma family of tumors is feasible and appeared to significantly improve the outcome for patients with high risk localized tumors 4
Surgical Planning and Imaging
- Both pre-chemotherapy and post-chemotherapy MRI scans should be taken into consideration for surgical planning in patients with localized Ewing's sarcoma who will undergo surgery 2
- In certain anatomic locations with good chemotherapy response, the post-chemotherapy MRI may be the appropriate imaging modality to plan surgical resection margins 2
Management of Relapsed Ewing Sarcoma
- The management of relapsed/refractory Ewing sarcoma (RR-ES) is complex and controversial, and requires a multidisciplinary approach 5
- Consensus recommendations for systemic therapies in the management of RR-ES include the use of molecular testing, chemotherapy regimens and novel agents, maintenance therapy, and high-dose chemotherapy with autologous stem cell rescue 5