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This article reports an unusual presentation wherein the first evidence of distant failure from a locally controlled, recurrent skull base chordoma was a metastasis to the mandible. We present a case report from a tertiary-care academic skull base referral center and a review of literature. A 33-year-old woman with a locally recurrent spheno-occipital chordoma that was stabilized with multimodality therapy presented with a right mandibular mass. Immunohistochemical stains demonstrated the tumor cells staining positive for vimentin, cytokeratin, epithelial membrane antigen (EMA), and S100 consistent with metastatic chordoma. Positron emission tomography-computed tomography imaging further revealed widespread distant failure. Chordomas are rare tumors with only four previous reports of metastasis to the mandible. This is the first presentation of a mandibular metastasis from a spheno-occipital chordoma. We present a review of literature and summarize the demographic, clinical, pathological, treatment-related data, and discuss follow-up information from previous reports of metastatic chordomas to the mandible.

Mandibular Metastasis from a Skull Base Chordoma: Report of a Case with Review of Literature