[Comprehensive treatment strategies for pediatric head and neck parameningeal rhabdomyosarcoma: a single-center retrospective analysis of 53 cases].

Abstract

Objective: To investigate the clinical characteristics and treatment outcomes of parameningeal rhabdomyosarcoma (PM-RMS) of the head and neck in children, and to analyze the risk factors influencing prognosis. Methods: Clinical data of children with PM-RMS admitted to Beijing Tongren Hospital of Capital Medical University between September 2009 and September 2023 were collected. The cohort included 27 males and 26 females, aged from 4 to 191 months. Overall survival and event-free survival rates were calculated using the Kaplan-Meier method. Log-rank test was used for univariate analysis and Cox regression model for multivariate analysis. Results: The predominant pathological subtypes were embryonal (30 cases, 56.6%) and alveolar (21 cases, 39.6%). There were 23 cases in stage Ⅱ, 16 cases in stage Ⅲ, and 14 cases in stage Ⅳ. According to risk stratification, there were 9 cases with low-risk, 24 with intermediate-risk, 8 with high-risk, and 12 with the invasion of central nervous system. The median follow-up time was 36 months (range: 4-152 months). The overall survival rate was 67.9% (36/53) and the 5-year overall survival rate was 53.6%. All patients received chemotherapy and 43 patients received radiotherapy. Univariate analysis revealed that lymph node metastasis (χ²=4.82, P=0.028), distant metastasis (χ²=8.63, P=0.003), and absent of radiotherapy (χ²=4.18, P=0.041) were significantly associated with poor prognosis. Multivariate analysis identified distant metastasis (HR=4.888, 95%CI: 1.345-17.769, P=0.016) and absent of radiotherapy (HR=5.155, 95%CI: 1.637-16.130, P=0.005) as independent risk factors for prognosis. Conclusion: Pediatric PM-RMS of the head and neck is highly malignant, prone to distant metastasis, and associated with a poor prognosis. Radiotherapy can improve local control rates in affected children.