Clivus chordoma in childhood

Abstract

A 9-year-old boy with mild left hemiparesis and left abducens nerve palsy was found to have chordoma of the clivus. Magnetic resonance imaging (MRI) was most useful not only for diagnosis but also for selection of operative approaches. The tumor was subtotally resected by a transoral-transpharyngeal approach followed by a retroauricular retromastoid craniotomy, and postoperative radiation therapy was administered. Intracranial chordomas are rare in children; only 12 cases have previously been reported in detail. Eleven patients were male and one was female. Progressive cranial nerve involvement and long tract signs without increased intracranial pressure constitute the cardinal symptomatology of intracranial chordomas, but a separation of cranial sutures and enlargement of the head may be seen in children. The role of MRI in diagnosis and patient management cannot be overemphasized.