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Chordoma is a rare bone tumor often present in the skull base and spine. In addition, it is not sensitive to radiotherapy that surgical resection is of great significance for the treatment of chordoma. Residual tumors that cannot be surgically removed usually lead to tumor recurrence. Studies have shown that chordoma will be accompanied by multiple gene mutations, such as PDGFR, EGFR, HER2, VEGFR, and mTOR, and interact with the host immune system to promote tumor progression. Targeted therapy and immunotherapy can improve the prognosis of chordoma patients to some extent. This review focuses on the clinical trials related to targeted therapy, immunotherapy, and chemotherapy of chordoma.
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