Pediatric and Young Adult Chordoma: A systematic Review and Meta-analysis

Introduction: Pediatric chordoma is a rare primary tumor that occurs most often in the skull base, spine, or sacrum. There is limited literature on a large cohort of pediatric chordoma patients and their treatment. The goal of this study is to combine the available literature to better elucidate the treatment modalities used in treating pediatric chordoma.

Methods: A systematic review was conducted using the search criteria “pediatric” and “chordoma” on Pubmed. The initial search yielded 234 studies. Studies that included patients with a chordoma confined to the spine, sacrum, or skull base younger than 30 years were included. Papers with mixed pathologies or pediatric and adult patients whose data could not be separated were excluded.

Results: From the initial 234 studies 18 were included yielding 499 total patients. Of these 470 (94.2%) were skull base, 20 (4%) were mobile spine, and 9 (1.8%) were sacral. There was a male to female ratio of 0.93, and a median age of 11 years ranging from 3 months to 30 years. Treatment modalities broke into 73 (14.6%) surgery, 326 (65.3) surgery + radiation, and 43 (8.6%) receiving radiation alone. Of patients who received surgery and papers that reported it (323 patients), 80 (24.8%) achieved gross total resection (GTR), while 243 (75.2%) had subtotal resection (STR). Of the studies that reported pathology (387 patients), 257 (66.4%), 54 (14.0%) and 76 (19.6%) were conventional, poorly dedifferentiated, and chondroid respectively.

Conclusion : Most cohort studies with cases of pediatric chordoma present skull base cases with patients receiving surgery and radiation. However, there is little evidence and much contention of what is best practice in managing pediatric chordoma. Many of these studies have inconsistent outcomes data or short follow up times. A large multicenter cohort needs to be created with consistently reported outcome data to help elucidate optimal treatment for these patients.