JAAOS - 2026-03-15 - Journal Article; Review
Contemporary Management of Standard Chordoma Arising in the Mobile Spine and Sacrum.
Mascarenhas L, Balzer B, Nguyen AT, Schwab JH
Topics
Key Takeaway
En bloc resection with negative margins remains the primary treatment for spinal and sacral chordoma, yet late local recurrence occurs in up to 50% of cases within 10–15 years despite oncologically adequate surgery.
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Summary
This narrative review addresses contemporary surgical, radiation, and systemic management of chordoma arising in the mobile spine and sacrum. En bloc resection targeting negative margins is the standard of care, with advanced radiation modalities (SBRT, proton therapy, carbon ion therapy) serving as adjuncts when margins are positive or surgery is not feasible. Despite aggressive local therapy, recurrence rates reach 50% at 10–15 years, and no systemic agent has demonstrated definitive disease control.
Key Limitation
The review provides no pooled recurrence, survival, or complication data stratified by margin status, radiation modality, or anatomic location, limiting its ability to guide specific treatment selection decisions.
Original Abstract
Chordomas are rare, slow-growing tumors originating from the notochord, commonly occurring in the sacrum and mobile spine. These tumors have a high rate of local recurrence and potential for metastasis. Local management continues to evolve. The primary treatment approach involves en bloc resection aiming for negative margins, but even with this aggressive strategy, late recurrences occur in up to 50% of cases within 10 to 15 years. Advanced radiation therapy techniques, such as stereotactic body radiation therapy, proton therapy, and carbon ion therapy, play an ever-increasing role as adjuvants to surgery, particularly when negative margins are not achieved, and, in select cases, as standalone treatment. Despite these advancements, chordomas remain challenging to treat due to their tendency for late recurrences and metastases. Emerging therapies such as immunotherapy provide hope for improved treatment with less morbidity. The management of chordoma requires a multidisciplinary approach integrating surgery, radiation therapy, and systemic therapies to optimize local control and long-term survival outcomes. Continued research and clinical trials are essential for improving treatment efficacy and developing novel therapeutic strategies for this locally aggressive tumor type.