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Sacral chordomas: surgical management, reconstruction, and translational advances: a systematic review
Vivek Sanker1, Sawsane Ghaddar2, Anam Sayed Mushir Ali3
1Department of Neurosurgery, Stanford University, Stanford, CA, USA.
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.
Insights
Sacral chordomas are rare tumors requiring complex surgery. Multimodal care combining advanced reconstruction, particle radiotherapy, and targeted therapies improves outcomes and local control for these challenging cancers.
Area of Science:
- Oncology
- Surgical Oncology
- Radiotherapy
Background:
- Sacral chordomas are rare, locally aggressive malignant tumors originating from notochordal remnants.
- They present significant surgical challenges due to indolent growth and high recurrence rates.
- Current treatment strategies aim to balance local control with functional preservation.
Purpose of the Study:
- To synthesize current evidence on the surgical management of sacral chordomas.
- To review modern reconstruction techniques and adjuvant therapies.
- To explore translational advances and molecular insights for improved patient outcomes.
Main Methods:
- Systematic review of literature from PubMed, Scopus, and Web of Science (2023-2025).
- Analysis of surgical management, reconstruction methods, adjuvant therapies, and systemic treatments.
- Inclusion of studies on molecular drivers and novel immunotherapeutic approaches.
Main Results:
- En bloc resection with wide margins is the primary treatment, though associated with morbidity.
- Advanced reconstruction (vascularized grafts, 3D-printed prostheses) enhances stability.
- Particle beam radiotherapy (proton/carbon ion) shows superior local control (77-89%) versus photon therapy (10-30%).
- Targeted therapies stabilize disease in 60-70%; immunotherapies are under investigation.
- Key molecular drivers include Brachyury (TBXT) and receptor tyrosine kinases.
Conclusions:
- Integrated multimodal care is essential for enhancing survival and function in sacral chordoma patients.
- Advances in surgical techniques, radiotherapy, and targeted therapies offer improved management options.
- Ongoing research into molecular targets and immunotherapy holds promise for future treatment strategies.
