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Ewing's sarcoma (EWS) is a highly malignant, small round cell tumor that predominantly arises in the pelvis and long bones of children and young adults. Its primary manifestation in the skull is exceptionally rare. Due to its atypical location diagnosis can be challenging and may mimic other skull-based lesions. Early recognition and a multidisciplinary approach are essential for optimal management and prognosis. Here, we present a case of a 28-year-old woman with a localized swelling over right fronto-temporal region and headache since 1 month, magnetic resonance imaging (MRI) revealed extra-axial mass lesion in the right frontotemporal region. A right frontotemporal craniotomy with gross total excision was done, the histopathological examination and immunohistochemistry revealed it to be EWS. The patient then received adjuvant chemotherapy with vincristine, doxorubicin, cyclophosphamide, and mesna protocol for 12 weeks followed by MRI that suggested postoperative changes. Subsequently local radiation therapy of 55.8 Gy in 31# was delivered. Currently patient is on chemotherapy. In this case report, we discuss the presentation of EWS in a rare location and it’s course of treatment.
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Thesiya et al. (2025) studied this question.
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