Background: Extra-axial chordomas are exceedingly rare malignant tumours. Herein, we present the first case of an extra-axial chordoma in the gingivae.
Methods and results: A 21-year-old man presented with a suspected chordoma in the upper right gingiva. Diagnosis was difficult; however, owing to strong expression of the chordoma marker brachyury, extra-axial chordoma was ultimately diagnosed. The tumour was completely resected without performing a facial incision. To ensure its safety and effectiveness, the surgical procedure was simulated several times before its performance using a three-dimensional (3D) model. Twenty-four months after surgery, the patient remains disease-free.
Conclusion: A diagnosis of extra-axial chordoma can be confirmed by immunohistochemical staining for brachyury.
Keywords: Bone tumour; Brachyury; Extra-axial chordoma; Surgical resection; Three-dimensional model.
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