Clivus chordoma in a 9-year-old child: case report and review of the literature

Abstract

Chordomas are bone tumors of the axial skeleton. They arise from notochordal remnants. In children these tumors are extremely rare and are predominantly located in the skull base. The authors report on a clivus chordoma in a 9 7/12-year-old girl. It presented as a nasopharyngeal mass with destruction of the clivus and paralyses of the ninth, tenth, and eleventh cranial nerves on the right side. After incomplete resection by a transoral transclival route, high-dose radiotherapy was added. This treatment was effective as demonstrated by follow-up CAT scans. A short review of the current literature is given. The local recurrence rate is extremely high, and distant metastases may occur. Complete resection is rarely possible, and combined management with postoperative radiotherapy is propagated. Permanent cure is rare, and at the present time, chemotherapy appears to be of no value in the primary treatment of chordomas.