J Neurol Surg B Skull Base 2016; 77 - FP-15-01
DOI: 10.1055/s-0036-1592513

Outcomes in the Treatment of Pediatric Skull Base Chordoma

Nathan T. Zwagerman 1, George Zenonos 1, Maria Koutourousiou 2, Joseph D. Chabot 1, Carl H. Snyderman 1, 3, Elizabeth C. Tyler-Kabara 1, Paul A. Gardner 1
  • 1Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States
  • 2Department of Neurological Surgery, University of Louisville, Louisville, Kentucky, United States
  • 3Department of Otolaryngology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States

Objective: Chordoma of the skull base in the pediatric population is rare. There is an absence in the literature regarding large treatment series in the pediatric population.

Methods: We retrospectively reviewed all pediatric patients with skull base chordomas at our institution from 2004 to 2015 and found 10 patients who underwent surgical treatment.

Results: Our series included 8 male and 2 female patients with a mean age of 12 years (range: 4–18). A total of 10 surgeries were performed on these patients and included predominantly an endoscopic endonasal approach (EEA). Two patients underwent treatment with chemotherapy. Four patients developed tumor progression. The average follow-up was 55.5 months (1.5–106 months). Complications included one carotid artery injury resulting in hemiparesis that improved on follow-up. Two patients had postoperative cranial nerve XII palsies; one of these was permanent, the other significantly resolved on follow-up. Four patients had postoperative cerebrospinal fluid (CSF) leaks that required further operations for treatment. One patient developed seeding of the inferior turbinate which was resected, and one patient presented with intradural cervical spine metastasis and developed another. One patient developed a postoperative hematoma requiring an evacuation.

Conclusion: Pediatric skull base chordoma is a rare entity that presents multiple challenges to treatment. A multispecialty team is required for proper treatment, but endoscopic endonasal approaches provide a good option for resection; long-term tumor control is possible with combined therapy. Pediatric patients are at increased risk for postoperative CSF leak.