RESUMO
BACKGROUND: Orbital rhabdomyosarcoma (RMS), the most common primary malignant orbital tumor in childhood, presents unique challenges in management due to its genetic basis and abnormal cellular proliferation. Management has evolved from surgical excision to multimodal approaches, including surgery, radiotherapy, and chemotherapy. This review explores trends in epidemiology, pathophysiological insights, and treatment evolution to delineate optimal therapeutic strategies. METHODS: A literature search was conducted across PubMed and Scopus databases up to March 4, 2024, using keywords related to orbital RMS. Studies were selected based on predefined criteria, focusing on clinical presentation, diagnostic modalities, management strategies, and outcomes. Data extraction and analysis were performed independently by two reviewers, with quality assessment based on the Oxford Centre for Evidence-Based Medicine 2011 guidelines and Joanna Briggs Institute checklists. RESULTS: The review synthesized data from 17 studies, highlighting demographic patterns, clinical characteristics, staging, and treatment approaches. Most patients were male and white, with a higher proportion of early-stages diagnoses. The embryonal subtype was the most common, and treatment has shifted towards radiation therapy and combined modalities. In orbital RMS, several modalities are applied in treatment. There are radiotherapy, surgical interventions, and chemotherapy. Recent studies put more emphasis on the long-term outcome of the disease about orbital bone development and facial symmetry. CONCLUSION: Management of orbital RMS has evolved significantly, with a current emphasis on multimodal treatment strategies. Future research should focus on optimizing therapeutic approaches to balance effective tumor control with the preservation of orbital function and aesthetics.
