Primary Intracranial Ewing Sarcoma With an Unusual Presentation: A Case Report

초록

Primary extraosseous intracranial Ewing sarcoma (ES) is an extremely rare disease, limited to the pedi- atric population, that primarily originates in the skull. Here, we present an unusual case of adult Ew- ing’s sarcoma originating from the brain parenchyma. The 50-year-old male patient visited our hospital with severe headache lasting 3 weeks. MRI presented 6.1×6.2×5.2 cm sized heterogeneously en- hanced mass containing peritumoral edema in the right frontal lobe. The patient underwent right frontal craniotomy, at which time the gray and red masses adhered to the surrounding brain parenchyma. The mass was completely resected using neuronavigation and electrophysiological monitoring. Histo- pathological examination revealed ES-compatible findings of small round cell tumor and CD-99 posi- tive membranous immunostaining. Next generation sequencing revealed translocation and fusion of EWSR1 and FLI1, consistent with a confirmed diagnosis of ES. Consequently, the patient underwent postoperative radiotherapy. The present case revealed adult primary intracranial ES arising from the frontal lobe. Although its etiology remains poorly understood, intraparenchymal ES should be included in the differential diagnosis of parenchymal brain tumors

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