Extracranial presentation of craniocervical chordoma

Chordomas are rare tumors of notochordal origin which arise in the sacral, vertebral, and craniocervical areas. Most patients with craniocervical chordomas have either neurological or ophthalmological symptoms. A patient presented with a parotid mass which was initially diagnosed as benign mixed tumor. One and one-half years later, this patient was found to have an extensive intracranial tumor which was diagnosed as chordoma. Treatment consisted of subtotal surgical excision and radiotherapy. This case demonstrates an unusual presentation of an unusual tumor and the difficulty that may arise in in diagnosing these tumors.