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

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.