Pediatric Head and Neck Chordomas: SEER Analysis of Tumor Location, Treatment Efficacy, and Survival Outcomes

Introduction: Chordomas are rare tumors, notochord-derived tumors predominantly affecting the skull base and spine. While more common in adults, their occurrence in pediatric populations, particularly in the head and neck, is gaining is of growing interest due to evidence suggesting distinct clinical behavior and potentially poorer prognosis. This study analyzes the epidemiological trends, treatment approaches, and survival outcomes in pediatric head and neck chordomas.

Methods: The Surveillance, Epidemiology, and End Results (SEER) database identified 3,566 primary chordomas from 2000 to 2021, with head and neck tumors account for 41%. Of these 1,468 cases, 141 occurred in pediatric patients ( < 19 years of age), while 1,327 were diagnosed in adults (> 20 years). Demographic and clinicopathologic characteristics, as well as survival outcomes, were analyzed for the pediatric cohort.

Results: Among 141 pediatric head and neck chordomas, the mean age was 10.6 years (SD, 5.10). The majority were White (80.1%) and female (53.2%). At diagnosis, most tumors were localized (50.4%), while 37.6% spread regionally and 12.0% were metastatic. Treatment approaches included surgery alone (31), radiation alone (1), and a combination of both modalities (52), while 57 cases lacked treatment data. The radiation-only group yielded the highest mean survival (16.3 years), followed by surgery alone (7.25 years) and combined therapy (5.88 years).

Conclusions: Pediatric head and neck chordomas showed a mean survival of 7.30 years, highlighting a poor prognosis likely due to resistance to standard therapies. While the primary treatment approach is surgery with adjuvant radiation, combined therapy in this cohort yielded the lowest mean survival, followed by surgery and radiation alone. The unusually high survival observed in the radiation-only group may reflect the small sample size, which consisted of a single patient. These findings suggest unique clinical behavior in pediatric head and neck chordomas, underscoring the need for focused research to refine therapeutic strategies for this rare population.