Outcomes in the Treatment of Pediatric Skull Base Chordoma

Introduction: Chordoma of the skull base in the pediatric population is rare. These tumors tend to be extremely aggressive and prone to recurrence. 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). The most common complaint was neck pain (n = 4) followed by swallowing difficulty (n = 3), double vision (n = 2) and nasal congestion (n = 2). A total of 10 surgeries were performed on these patients and included predominantly an endoscopic endonasal approach (EEA). Two of these surgeries were staged and two others were combined with transcervical approaches. One patient had a prior biopsy with upfront radiation and one patient had a previous resection before presentation at our institution. Six patients underwent postoperative proton beam therapy. Two patients underwent treatment with chemotherapy. Four patients developed tumor progression. Two of these underwent further resection, and two patient’s family elected to stop treatment. Six patients were recurrence free upon follow up. The average follow up was 55.5 months (range 1.5–106 months). Complications included one carotid artery injury resulting in hemiparesis that improved on follow up. Two patients had post-operative cranial nerve XII palsies. One of these was permanent, the other significantly resolved on follow up. Four patients had post-operative 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 post-operative hematoma requiring an evacuation.

Conclusions: 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.