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.
Mascarenhas et al. (Tue,) studied this question.