Abstract
BACKGROUND: Sacral chordomas are rare malignant tumors arising from notochordal remnants that present unique surgical challenges due to their indolent growth, locally aggressive behavior, and high recurrence rates despite treatment. This review synthesizes current evidence on surgical management, reconstruction techniques, adjuvant therapies, and translational advances for sacral chordomas. METHODS: This systematic review synthesizes current evidence on surgical management, reconstruction techniques, adjuvant therapies, and translational advances through analysis of PubMed, Scopus, and Web of Science databases [2023-2025]. RESULTS: En bloc resection with wide negative margins remains the cornerstone of treatment, achieving optimal local control but often at the cost of significant neurological and functional morbidity. Modern reconstruction techniques, including vascularized grafts and three-dimensional (3D)-printed prostheses, have improved spinopelvic stability and postoperative outcomes. Adjuvant particle beam radiotherapy, particularly proton and carbon ion therapy, demonstrates superior 5-year local control rates of 77-89% compared to 10-30% with conventional photon therapy, while stereotactic radiosurgery achieves 81% local control. Systemic therapies remain limited, though targeted inhibitors of platelet-derived growth factor receptor (PDGFR), epidermal growth factor receptor (EGFR), and the PI3K/AKT/mTOR pathway show modest activity with disease stabilization in 60-70% of cases. Novel immunotherapeutic approaches, including Brachyury-targeted vaccines and checkpoint blockade, are under investigation. Molecular insights have identified key drivers, including Brachyury [T-box transcription factor T (TBXT)], receptor tyrosine kinases, and emerging biomarkers that may guide future therapeutic selection. CONCLUSIONS: Integrated multimodal care is critical for improving survival and function in patients with sacral chordomas.