Pediatric Head and Neck Chordomas: A National Analysis of Prognostic Indicators and Survival Outcomes

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OBJECTIVE: To identify prognostic factors among pediatric patients with head and neck chordomas. STUDY DESIGN: Cross-sectional analysis. SETTING: The National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) 22 database. METHODS: Pediatric patients (<19 years of age) with head and neck chordomas were identified using the SEER database, spanning the years 2000 through 2021. Demographics and clinicopathologic characteristics were assessed using descriptive statistics and stratified by age (<10 years vs ≥10 years). Overall survival (OS) and cancer-specific survival (CSS) were estimated using Kaplan-Meier analysis, and Cox proportional hazards models were used to evaluate the associations with survival. RESULTS: Among the 141 cases, 57.4% were aged ≥10 years. Age ≥10 years was associated with improved OS and CSS (adjusted HRs 0.26 and 0.14, respectively) Most tumors were conventional chordomas (85.8%) located in the skull base (84.4%). Dedifferentiated and chondroid subtypes were more common in patients <10 and ≥10 years, respectively, and both were associated with significantly worse CSS (adjusted HRs 8.06 and 6.36, respectively). At diagnosis, 41.8% had localized disease, while 9.9% had distant metastases. The most common treatment was surgery with adjuvant radiation (40.7%). Definitive chemotherapy was a risk factor for worse OS (HR 8.99) and CSS (HR 11.67). CONCLUSION: Pediatric head and neck chordomas primarily develop at the skull base, with survival outcomes influenced by factors such as age, tumor histology, and treatment approach. Younger age, nonconventional histology, and chemotherapy were associated with poorer prognosis, highlighting the importance of age-appropriate, multidisciplinary management strategies.

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