Ewing sarcoma – current diagnostic and therapeutic approaches

BACKGROUND: Ewing sarcoma belongs to the family of undifferentiated small round cell sarcomas of bone and soft tissue. It is characterized by a gene fusion involving EWSR1 and an ETS-family transcription factor gene. In 85-96% of cases, a specific chromosomal translocation results in the EWSR1-FLI1 fusion gene, whose product functions as an oncogene essential for tumorigenesis. Ewing sarcoma is most common in adolescents and young adults. It primarily affects the diaphyses of long bones, the pelvis, and the axial skeleton, although extraosseous involvement is not uncommon. This is a highly malignant neoplasm, and in most cases, micrometastases are already present at the time of diagnosis. The treatment is multimodal and includes local therapy (surgery and/or radiotherapy) and systemic chemotherapy. One of the greatest therapeutic challenges remains the long-term systemic control of the disease. To improve overall survival - especially in high-risk patients - innovative treatment strategies are essential, as the potential for intensifying chemotherapy has reached its limit due to treatment-related toxicity. Both diagnostic and therapeutic management should take place in specialized sarcoma centers. AIM: This article aims to provide an up-to-date overview of current diagnostic and therapeutic approaches in Ewing sarcoma.