Ewing's sarcoma (ES) is a malignant small round cell neuroectodermal tumour primarily affecting children in the first and second decade of life. Since ES is difficult to control, early diagnosis is crucial, and the treatment requires a multimodality approach constituting chemotherapy, surgery and radiotherapy. Here, we present a case of ES of orbit in a 17-year-old female diagnosed in 2021. This case report focuses on the clinicopathological presentation and management principles of this rare manifestation of the disease that unilaterally infiltrated into the extraconal space of the orbit as well as the I/L frontal sinus. The patient underwent chemotherapy and radiation, and she is on follow-up.
Keywords: ewing's sarcoma; ewsr1 translocation; nkx2.2; orbit; round cell tumour.
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