Abstract
BACKGROUNDChordomas are rare malignant neoplasms that develop from the primitive notochord with < 5% of the tumors occurring in pediatric patients younger than the age of 20. Of these pediatric chordomas, those affecting the craniocervical junction (C1–C2) are even more rare; therefore, parameters for surgical management of these pediatric tumors are not well characterized.OBSERVATIONSIn this case, a 3-year-old male was found to have a clival chordoma on imaging with extension to the craniocervical junction resulting in spinal cord compression. Endoscopic-assisted transoral transclival approach for clival tumor resection was performed first. As a second stage, the patient underwent a left-sided far lateral craniotomy and cervical laminectomy for resection of the skull base chordoma and instrumented fusion of the occiput to C3. He made excellent improvements in strength and dexterity during rehab and was discharged after 3 weeks.LESSONSIn pediatric patients with chordoma with extension to the craniocervical junction and spinal cord compression, decompression with additional occipito-cervical fusion appears to offer a good clinical outcome. Fusion performed as a separate surgery before or at the same time as the initial tumor resection surgery may lead to better outcomes.
Highlights
Chordomas are rare malignant neoplasms that develop from the primitive notochord with < 5% of the tumors occurring in pediatric patients younger than the age of 20
These neoplasms mostly occur in adults between the ages of 30 and 70 whereas only
The authors describe the case of a 3-year-old male patient with a clival chordoma with extension to the craniocervical junction who underwent resection using a multistaged surgical approach
Summary
Chordomas are rare malignant neoplasms that develop from the primitive notochord with < 5% of the tumors occurring in pediatric patients younger than the age of 20. Evidence is limited to case reports of OCF after clival chordoma resection in the pediatric population, with no definitive guidelines provided.[11] the authors describe the case of a 3-year-old male patient with a clival chordoma with extension to the craniocervical junction who underwent resection using a multistaged surgical approach. He underwent OCF with a good postoperative outcome
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