Ewing sarcoma genomics and recent therapeutic advancements

Abstract

Ewing sarcomas are highly heterogenous mesenchymal tumors that develop in bone or soft tissue and primarily affect children, teenagers, and young adults. After osteosarcoma, it is the second most common malignant bone sarcoma. Ewing sarcoma, metastatic and relapsed, typically have a poor prognosis and recurrences are frequent, leading to high rates of morbidity and mortality. The ongoing inability to increase overall survival for patients with ewing sarcoma emphasizes the critical requirement for the quick translation of emerging therapy approaches. Targeting the EWR1/FLI1 fusion protein, which is the primary genetic anomaly and master regulator of ewing sarcoma and found in 80–90% of instances of ewing tumors, is the most crucial objective. This review offers new insights into the genomics and proteomics of ewing sarcoma signaling and how it influences the tumor microenvironment and disease progression. It also elucidates how recent technological advancements have explained some of the underlying oncogenic characteristics of ewing sarcoma. The current review examined existing and potential experimental therapies that, by enhancing patient survival and quality of life, target multiple signaling pathways involved in the progression of ewing sarcoma. These therapies may one day replace current regimens as the new standard of care for patients.

Keywords

• Ewing sarcoma
• Genomics
• Tumor
• Immunotherapy
• Fusion protein