Head and Neck Rhabdomyosarcoma in Children: An Otolaryngological Perspective on Diagnostic and Therapeutic Approaches

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Abstract Purpose of Review Head and neck rhabdomyosarcoma (HNRMS) is the most common soft-tissue sarcoma in children, with approximately 30–40% of cases arising in the head and neck region. Due to its anatomical complexity, histopathological diversity, and molecular heterogeneity, HNRMS remains a major diagnostic and therapeutic challenge in pediatric otolaryngology. This review aims to summarize current evidence regarding the epidemiology, clinical presentation, diagnostic strategies, contemporary treatment modalities, and long-term complications associated with pediatric HNRMS. Purpose of Review. Recent Findings Recent advances in molecular pathology have significantly improved risk stratification in rhabdomyosarcoma, particularly through the identification of FOXO1 fusion status as a key prognostic marker. Modern imaging techniques, especially magnetic resonance imaging and PET-based assessment, have enhanced diagnostic precision and evaluation of local tumor extension. Contemporary multimodal treatment protocols combining chemotherapy, conservative surgery, and proton beam radiotherapy have improved survival outcomes while reducing treatment-related morbidity. Furthermore, growing attention has been directed toward survivorship issues, including craniofacial deformities, endocrine dysfunction, hearing impairment, and secondary malignancies. Summary The prognosis of pediatric HNRMS depends strongly on tumor location, histological subtype, and molecular characteristics. Orbital tumors are associated with favorable outcomes, whereas parameningeal lesions continue to pose substantial therapeutic difficulties because of delayed diagnosis and proximity to critical anatomical structures. Early recognition, accurate histopathological and molecular diagnosis, and individualized multidisciplinary treatment are essential to optimize survival and minimize long-term complications. Ongoing developments in targeted therapy and proton radiotherapy may further improve both oncological outcomes and quality of life in affected children. This review is important because it integrates current molecular, diagnostic, and therapeutic advances in pediatric HNRMS from an otolaryngological perspective, highlighting both survival outcomes and long-term quality-of-life considerations. Moreover, it emphasizes the need for early multidisciplinary management in anatomically complex head and neck tumors, which remains crucial for improving prognosis and reducing treatment-related morbidity.