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This report presents the case of a 9-year-old girl with an initial diagnosis of bilateral orbital tumor, referred for severe proptosis and tumor-like ocular lesions.Although retinoblastoma was initially suspected, clinical and paraclinical studies revealed findings compatible with granulomatosis with polyangiitis, a rare autoimmune inflammatory disease in pediatrics.This report highlights the value of an interdisciplinary approach to differentiate neoplastic processes from inflammatory and autoimmune etiologies, with a focus on ocular preservation and systemic management.Collaboration between ophthalmologists, rheumatologists, pediatric oncologists, and pathologists was crucial to guide diagnosis and management.Finally, this report emphasizes the importance of investigating rare diseases and maintaining a comprehensive approach that protects the patient while clarifying the diagnosis, optimizing the clinical approach, and avoiding unnecessary interventions.
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León et al. (2026) studied this question.
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