Molecular Characteristics, Diagnostic and Therapeutic Strategies, and Future Directions of Undifferentiated Pleomorphic Sarcoma

Undifferentiated Pleomorphic Sarcoma (UPS), the most morphologically and genetically heterogeneous soft tissue sarcoma (STS) subtype, poses persistent challenges in clinical diagnosis and treatment. Its aggressive biology and resistance to conventional therapies stem from complex genomic variations, driver mutations, epigenetic dysregulation, and tumor microenvironment (TME) heterogeneity. This review systematically summarizes UPS's molecular genetic features, diagnostic/differential diagnostic approaches, current treatment paradigms, and emerging therapeutic directions—with a focus on immune checkpoint inhibitors, targeted therapy, and multi-omics-guided precision medicine. Integrating existing literature and latest clinical trial data, we provide a theoretical basis for UPS clinical management and prospect future research. Despite ongoing challenges, deepened understanding of tumor heterogeneity and novel therapeutic models are advancing the era of precision diagnosis and treatment for UPS.