From the Guidelines
The primary treatment approach for Ewing's sarcoma should involve a combination of chemotherapy, surgery, and/or 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's sarcoma, the goal is to achieve the best possible outcome in terms of morbidity, mortality, and quality of life.
- The treatment plan typically includes a standardized chemotherapy regimen, which is the same for patients with localized and metastatic disease 1.
- For local control, surgery is preferred, with an attempt to achieve a wide surgical margin, and radiation therapy (RT) is given to patients with marginal or intralesional surgery or with inoperable tumors 1.
- The radiation dose depends on the tumor site, with 40–45 Gy recommended for microscopic residual disease and 50–60 Gy for macroscopic disease 1.
- In cases of metastatic disease, total lung irradiation should be considered for patients with lung metastases who achieve a complete remission, and thoracotomy should be evaluated for patients with limited residual macroscopic disease 1.
- Follow-up care is crucial, with patients being followed at 3-month intervals until 3 years after the end of treatment, then at 6-month intervals until 5 years, and at 8–12 month intervals until at least 10 years 1. Key considerations in the treatment of Ewing's sarcoma include the risk of late relapse, long-term toxicity to heart, kidneys, and lungs if irradiated, and a 5% risk for second cancers 1.
From the Research
Overview of Ewing's Sarcoma
- Ewing's sarcoma is a rare and malignant tumor of bone and soft tissue that primarily affects adolescents and young adults 2, 3, 4, 5.
- The standard treatment for Ewing's sarcoma involves a multidisciplinary approach, including multiagent induction chemotherapy, 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.
Treatment Options
- The treatment of Ewing's sarcoma may include:
- Chemotherapy: multiagent induction chemotherapy, such as vincristine, doxorubicin, and cyclophosphamide (VAdriaC), followed by additional chemotherapy 2, 6.
- Surgery: local treatment with surgery, which may be combined with radiation therapy 2, 6.
- Radiation therapy: definitive radiation, which may be used as the primary approach to local control 2, 6.
- Experimental therapies, such as targeted therapy and high-dose chemotherapy with autologous stem cell transplantation, are also being explored 3, 4.
Prognosis and Challenges
- The prognosis for patients with Ewing's sarcoma is generally poor, especially for those with metastatic or recurrent disease 4, 5.
- The treatment of Ewing's sarcoma is associated with significant morbidity, including febrile neutropenia, cardiac dysfunction, and secondary myelodysplastic syndrome 6.
- International collaborations have defined the current standard of care, but there is still a need for further research to improve treatment outcomes and reduce treatment-associated morbidities 3, 5.