Comparative Analysis of Characteristics and Survival Outcomes in Pediatric Patients with Spinal Chordomas: Insights from the National Cancer Database and Systematic Review of the Literature

Introduction: Spinal chordomas are rare tumors with significant management challenges. As this pathology occurs more frequently in adults, presentation and outcomes in pediatric patients remains understudied. We aim to analyze and contrast the clinical characteristics, demographic variables, and survival outcomes of pediatric and adult populations with spinal chordomas. This study utilizes data from the National Cancer Database (NCDB) to address the gap in research on pediatric spinal chordomas and to explore these variations between age groups.

Methods: A systematic search of the literature was performed on MEDLINE and Web of Science from inception to March 2024 using the keywords “spinal,” “chordoma,” and “pediatric.” Additionally, the NCDB was queried for pediatric patients (≤ 21 years) with spinal chordoma treated between 2004 and 2017. These were compared to adult patients with spinal chordoma. Baseline characteristics, tumor specifics, treatment details, and survival outcomes were collected and analyzed.

Results: From the literature, 45 pediatric chordoma patients were identified, with a median age of 7 years. Most chordomas were in the cervical spine (40%), and 93% of the patients received surgical treatment. Gross total resection was achieved in 59% of cases, and 49% received adjuvant radiotherapy. Recurrence, metastasis, and mortality rates were 7%, 18%, and 24%, respectively at a median follow-up of 12 months. In the NCDB cohort, 53 pediatric patients (≤ 21 years) and 980 adults (> 21 years) were compared. Pediatric patients presented with more advanced tumors despite smaller sizes, with a higher proportion of stage 4 tumors. The chordomas were more commonly in the mobile spine (83% vs. 51%) and patients traveled further for treatment (57 vs. 27 miles). Pediatric patients also received higher radiation doses (5420 vs. 5049 cGy) compared with adults. Surgical resection and adjuvant radiotherapy were common treatments in both groups. After matching, outcomes, including survival rates and early mortality, were similar between age groups. Kaplan-Meier analysis showed no difference in overall survival probabilities between the age groups both prior to and after matching.

Conclusion : While pediatric patients with spinal chordomas present with more advanced stage tumors, they demonstrate similar overall survival outcomes when compared to adults.