From the Guidelines
Ewing sarcoma is a rare and aggressive type of cancer that forms in bones or soft tissues, typically affecting children and young adults, and requires a multidisciplinary approach for treatment, including chemotherapy, surgery, and radiation therapy. The disease is characterized by its rapid growth and potential to invade and destroy healthy body tissue, with common symptoms including pain, swelling, and tenderness at the tumor site, fatigue, fever, and unexplained weight loss 1.
Key Characteristics of Ewing Sarcoma
- It typically develops in the long bones of the legs and arms, the pelvis, chest wall, spine, or skull, though it can occur in any bone or soft tissue
- The disease begins when a cell in the body develops changes in its DNA, causing the cell to grow and multiply rapidly, forming a mass of abnormal cells
- Ewing sarcoma is a radiosensitive tumor, but surgery is the preferred treatment for local control, with a wide surgical margin attempted and radiation therapy given to patients with marginal or intralesional surgery or with inoperable tumors 1
Treatment and Follow-up
- Treatment usually involves a combination of chemotherapy, surgery, and radiation therapy, with standard chemotherapy regimens often including drugs such as vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide
- Patients with metastatic disease at diagnosis should have the same standardized chemotherapy as patients with localized disease, and total lung irradiation should be considered for patients with lung metastases who achieve a complete remission 1
- Follow-up care is crucial, with patients recommended to be 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, due to the risk of late relapse and long-term toxicity 1
From the Research
Definition and Overview of Ewing Sarcoma
- Ewing sarcoma is a malignant primary bone tumor that often involves soft tissue, affecting not only children but also young adults 2.
- It is the second most common primary malignant bone tumor in children and adolescents, accounting for 10% of primary malignant bone tumors in children and 3% of all childhood malignancies 2.
- Ewing sarcoma is a highly aggressive bone and soft-tissue tumor with peak incidence among adolescents and young adults 3.
Symptoms and Treatment
- The most common presenting symptoms of Ewing sarcoma are pain or swelling 2.
- Treatment for Ewing sarcoma consists of a multimodal approach, including chemotherapy, radiation therapy, and surgery 2.
- Chemotherapy regimens such as vincristine, doxorubicin, and cyclophosphamide (VAdriaC) and ifosfamide and etoposide (IE) have been used to treat Ewing sarcoma, with varying degrees of success 4, 5, 6.
Prognosis and Outcomes
- The prognosis for Ewing sarcoma patients varies depending on factors such as the presence of metastases, tumor size, and location 4, 5, 6.
- Patients with localized tumors tend to have better outcomes than those with metastatic disease 4, 5, 6.
- The 5-year overall survival rate for adults with localized Ewing sarcoma has been reported to be around 68-79% 5, 6.