Pediatric Sinonasal Rhabdomyosarcoma: Clinical Characteristics and Surgical Role

Abstract

Introduction: Rhabdomyosarcoma is the most common pediatric soft tissue sarcoma. It represents 5-8% of pediatric tumors. Head and neck is considered the most common site of RMS origin (40%). Parameningeal, orbital and non parameningeal RMS approximately represent 50%, 25% and 25% respectively. Histopathological evaluation demonstrates small round cells with high cytological variability which stain positive with desmin, myogenin and MyoD1. The mainstay treatment of RMS depends on chemotherapy and radiation therapy with a limited role of surgery. Method: A retrospective chart review for pediatric sinonasal rhabdomyosarcoma diagnosed and managed at King Fahad Specialist Hospital, Dammam, Saudi Arabia, and a literature review of pediatric sinonasal rhabdomyosarcoma was conducted. Results: A total of four cases were identified for the period (2011-2017), and a thorough review of their medical records and radiological imaging were done. Conclusion: Pediatric sinonasal rhabdomyosarcoma may initially present with symptoms mimicking rhinosinusitis. Biopsy and histological evaluation are the most essential steps to exclude malignancy. Intergroup Rhabdomyosarcoma Studies (IRS) established a staging system based on tumor extension and resectability. The role of surgery in RMS may be limited for obtaining biopsies for diagnosis and for palliative purposes. Popular antineoplastic agents used to treat RMS include vincristine, cyclophosphamide, actinomycin D, and adriamycin. Chemotherapy with alkylating agents has achieved a relapsed free survival 90% for nonparameningeal tumors and 65% for parameningeal tumors.