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Objective: We present the case of a 40-year-old female patient with an orbital pleomorphic rhabdomyosarcoma (RMS).Observations: Primary orbital RMS occurs before the age of 16 and there is a slight predilection between men and women.The incidence in adulthood is uncommon.Importance: This case highlights the importance of considering rare diagnoses in clinical practice, which is crucial for an adequate approach and rapid treatment.
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Fonseca-Aguirre et al. (2026) studied this question.
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