Chordoma of cervical vertebra protruding into the oral cavity

A 70-year-old man was admitted to hospital because of oral and cervical masses. Computed tomographic scanning revealed a lobulated mass lesion in the retropharyngeal region, with a protruding extension in the oral cavity and with destruction of the second cervical vertebra. A biopsy was performed under the diagnosis of a retropharyngeal tumor. Histologically, this lesion was composed of vacuolated tumor cells in a solid or cord-like arrangement, with an abundant myxoid matrix. Immunohistochemically, the tumor cells were positive for pancytokeratin, epithelial membrane antigen and S-100 protein. The tumor was diagnosed as chordoma. Chordoma presenting as an intra-oral mass lesion is very rare.