SA1 - Determinants of Recurrence, Metastasis, and Survival in Pediatric Head and Neck Sarcomas: The Role of Surgical Strategy, Margins, and Disease Burden

Abstract: Purpose: Head and neck sarcomas (HNS) in pediatric patients are rare, histologically heterogeneous malignancies that often arise in close proximity to critical structures. Their management requires balancing oncologic control with preservation of function, growth, and form. Pediatric-focused data spanning histologic subtypes and anatomic regions remain limited. This study evaluated oncologic outcomes, recurrence patterns, and surgical burden among pediatric patients with head and neck sarcoma treated at a single tertiary children's hospital.

Methods: A retrospective review was performed of pediatric patients treated for biopsy-proven head and neck sarcoma at a single tertiary children's hospital from January 1, 1995 through December 31, 2025. Patients with benign tumors, non-sarcomatous malignancies, tumors outside the head and neck, or follow-up < 6 months were excluded. The primary analysis was also done excluding fibrosarcoma cases due to their distinct biologic behavior and treatment paradigm. Demographic, tumor, treatment, and outcome variables were collected, including age, sex, tumor site, grade, tumor size, lymph node involvement, metastatic disease at presentation, margin status, and treatment sequencing. Tumors were grouped anatomically into lower face, midface, upper face, skull/scalp/neurocranium, neck, and multicompartment disease; a separate parameningeal subset analysis was also performed. Primary outcomes were recurrence-free survival (RFS) and overall survival (OS). Secondary outcomes included recurrence, death, and re-resection. Kaplan-Meier, log-rank, Cox proportional hazards, and logistic regression analyses were performed.

Results: Sixty patients met criteria for the primary analytic cohort; 25 (41.7%) had parameningeal involvement. Kaplan-Meier analysis demonstrated overall 1-, 3-, and 5-year RFS of 91.6%, 65.3%, and 56.6%, respectively, with a median RFS of 108.1 months. Corresponding 1-, 3-, and 5-year OS were 98.3%, 78.2%, and 71.9%; median OS was not reached. On univariate Cox analysis, metastatic disease was associated with significantly worse RFS (HR 3.47, 95% CI: 1.56 to 7.68, P = .0022) and OS (HR 4.22, 95% CI: 1.65 to 10.83, P = .0027). In multivariable Cox modeling, metastatic disease remained the only independent predictor of worse RFS and OS. Logistic regression likewise demonstrated that metastatic disease predicted recurrence (OR 11.26, P = .0187) and death (OR 9.10, P = .0275). In exploratory secondary analyses, a history of a genetic predisposition syndrome (OR 10.74, P = .0337) and neoadjuvant therapy (OR 4.46, P = .0129) were associated with recurrence, while parameningeal involvement was associated with increased odds of death (OR 3.14, P = .0498) and decreased odds of re-resection (OR 0.22, P = .0326). The multicompartment subgroup (n = 13) demonstrated poor long-term outcomes descriptively, with 5-year RFS and OS of 46.2% and 67.7%, respectively, although regression estimates were unstable because of sparse events. In the parameningeal subgroup (n = 25), 5-year RFS and OS were 48.0% and 59.5%; male sex was associated with worse OS (multivariable HR 5.35, 95% CI: 1.11 to 25.68, P = .0363).

Conclusions: Pediatric head and neck sarcomas carry substantial long-term oncologic risk, with recurrence accumulating over time despite relatively preserved early survival. Metastatic disease at presentation was the most consistent adverse prognostic factor across survival and event-based analyses. Parameningeal disease was associated with worse mortality and lower likelihood of re-resection, suggesting a particularly high-burden anatomic subset in which salvage options may be limited. These findings underscore the need for multidisciplinary treatment strategies that integrate oncologic control with thoughtful surgical and reconstructive planning in children with head and neck sarcoma.