Multi-institutional Pediatric Skull Base Chordoma Experience

Background: Chordomas are a rare malignancy in the pediatric population, and those occurring in the skull base are even rarer. Traditionally, chordomas are considered locally aggressive tumors with a high risk of recurrence and poor survival rates. Most centers see few cases, lending to the paucity of literature examining long-term outcomes of this disease, as well as the difficulty in establishing a standard of care in these patients.

Objectives: This study aims to report the combined experience of multiple institutions where pediatric skull base chordomas are treated to better understand this disease and identify trends in management.

Methods: We retrospectively reviewed patients age 18 and under with skull base chordomas at three tertiary care centers from 2004 to 2020. Information including demographics, clinical presentation, tumor characteristics, disease management, and treatment outcomes were collected and analyzed.

Results: Twenty-nine patients were identified with a mean age at diagnosis of 12 years old (range: 4–18 years). The most common presenting symptoms were diplopia (n = 10), followed by headaches (n = 9) and dysphagia (n = 5). All patients underwent surgery via endoscopic endonasal approach (EEA; n = 20), open craniotomy (n = 1), or combined endonasal and transcervical approaches (n = 7). Extent of resection was calculated in 23 patients, of whom 20 had near or gross total resection (≥95% resected). The most common surgical complication was CSF leak (n = 12). Twenty-two patients underwent surgery and radiation. Of those that underwent radiation, four received radiation preoperatively and the remainder received radiation postoperatively. The majority received proton beam therapy (n = 17), while two received photon therapy alone, and two underwent both photon and proton beam radiation. Four patients received chemotherapy. Of these, one received chemotherapy after resection of recurrence, one received chemotherapy after re-resection for progression, and two underwent chemotherapy alone for progression of disease. Average follow-up was 51 months (range: 1–136 months). Four patients suffered local recurrences, four had both local recurrence and metastases, and two patients had metastatic disease without local recurrence. Six patients died of disease. Average time-to-recurrence was 19 months (range 1–68 months). Subtotal resection had clear trends toward recurrence (p = 0.15) and death (p = 0.067). K_i-67 ≥ 15% was significantly associated with recurrence (p = 0.05) and death (p = 0.007). Receiving chemotherapy was also significantly associated with death (p = 0.006). Radiation treatment was not associated with differences in recurrence (p = 0.61) or death (p = 0.99). Patients who underwent endonasal resection had rates of recurrence (p = 0.99) and death (p = 0.57) that were similar to those who underwent open or combined approaches. Patients with exclusively paraclival chordomas had rates of recurrence (p = 0.99) and death (p = 0.99) that were similar to those with chordoma extending to other regions.

Conclusion: Multimodality therapy in the form of surgical resection and radiation seems to be the trend for treatment of pediatric skull base chordomas. Our findings suggest that subtotal resection and K_i-67 ≥ 15% are associated with poorer outcomes. Efforts to compile data from additional centers are underway and will be ideal in further investigating characteristics of this disease and identifying optimal treatment strategies.

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
12 February 2021