Giant “panfacial” chordoma involving skull base with intracranial invasion

Chordomas are rare, low-grade, primary malignant bone tumor arising from primitive notochord remnants of the axial skeleton. They rarely occur in an extraosseus craniofacial location such as nasopharynx, nasal septum, paranasal sinuses, and orbits. We report here an extreme case of "panfacial" chordoma involving middle and anterior skull base with intracranial invasion, which reached considerable size because of a delay in diagnosis and treatment. In this case, a wide surgical excision was performed to improve quality of life.