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Risk Factors for Survival in Pediatric Maxillofacial Rhabdomyosarcoma: A Single-Center Retrospective Cohort Study.

Ge Zhang1, Shengcai Wang2, Guoxia Yu3

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Journal of Oral and Maxillofacial Surgery : Official Journal of the American Association of Oral and Maxillofacial Surgeons
|April 6, 2026
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Summary

Pediatric maxillofacial rhabdomyosarcoma (RMS) outcomes are linked to chemotherapy response and tumor biology. Early response to neoadjuvant chemotherapy is a key factor for overall survival (OS) and event-free survival (EFS) in children with this rare cancer.

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Area of Science:

  • Pediatric Oncology
  • Head and Neck Cancer Research
  • Rhabdomyosarcoma Subtypes

Background:

  • Maxillofacial rhabdomyosarcoma (RMS) is a rare pediatric head and neck cancer with complex anatomical challenges.
  • Accurate risk stratification and treatment for maxillofacial RMS are hindered by its poorly defined nature and site overlap.

Purpose of the Study:

  • To determine the 5-year overall survival (OS) and event-free survival (EFS) rates in pediatric patients diagnosed with maxillofacial RMS.
  • To identify significant risk factors influencing OS and EFS in this patient population.

Main Methods:

  • Retrospective cohort study including pediatric patients (<18 years) with primary maxillofacial RMS.
  • Survival analyses utilized the Kaplan-Meier method and Cox proportional hazards regression.
  • Evaluated demographic, tumor, and treatment factors as potential predictors of survival outcomes.

Main Results:

  • The study included 46 pediatric patients with a median follow-up of 51.4 months.
  • Five-year OS and EFS rates were 70.2% and 66.0%, respectively.
  • Lack of response to neoadjuvant chemotherapy (SD/PD) and positive FOXO1 fusion status were significant independent risk factors for poorer outcomes.

Conclusions:

  • Pediatric maxillofacial RMS is a distinct subgroup with an intermediate prognosis compared to other head and neck sites.
  • Treatment outcomes are significantly impacted by early chemotherapy response and intrinsic tumor biology.
  • Identifying and addressing these factors is crucial for improving survival in pediatric maxillofacial RMS.