Outcomes of Pediatric Maxillofacial Giant Cell Lesion Management in Syndromic Versus Nonsyndromic Patients: A 21-Year Review.

Abstract

Background: Maxillofacial giant cell lesions (GCLs) may occur in isolation or as a part of a genetic syndrome, where they are often multifocal. The functional deficits and psychosocial impact necessitate urgent treatment; however, a consensus on management is lacking given the rarity and the variable presentation in children.

Purpose: This study aims to compare the treatment and outcomes of pediatric maxillofacial GCLs in syndromic and nonsyndromic subjects.

Study design, setting, and sample: A retrospective cohort study of pediatric subjects with histologically confirmed maxillofacial GCLs at a tertiary children's hospital between 2003 and 2024 was performed. Patients with incomplete documentation and less than 6 months of follow-up were excluded.

Predictor variable: The predictor variable was syndromic diagnosis.

Main outcomes variable(s): The primary outcome was tumor recurrence. The secondary outcome was final disease status (remission, progressive, or nonprogressive lesion).

Covariates: Demographic characteristics including syndromic diagnosis, tumor characteristics, and adjuvant pharmacologic therapy (APT), including duration of treatment and side effects, were collected. Lesions were classified as aggressive or nonaggressive according to Chuong et al. ANALYSES: Univariate and bivariate statistics were used to compare treatment characteristics and outcomes between syndromic and nonsyndromic cohorts, with statistical significance determined by P values less than .05. Time to tumor recurrence was estimated using Kaplan-Meier analysis.

Results: The sample was composed of 28 subjects (16 nonsyndromic, 12 syndromic), with a mean age of 10.7 ± 4.8 years and 17 (60.7%) were male. Overall, 96.4% of lesions were aggressive. Nonsyndromic subjects were more frequently treated with APT compared to syndromic subjects (75.0 vs 25.0%, P = .020). Recurrence occurred in one nonsyndromic subject (6.2%) and 50% of syndromic subjects (P = .008). The estimated median time to recurrence was 89 weeks. Remission was achieved in 100% of the nonsyndromic cohort and only 8.3% of the syndromic cohort (P < .001).

Conclusion: The results of this study demonstrated that syndromic subjects were less likely to receive APT for the management of pediatric maxillofacial GCLs, and exhibited higher recurrence and lower remission rates compared to their nonsyndromic counterparts. These findings emphasize the importance of long-term surveillance and anticipatory counseling for families of syndromic patients.