Solitary intracranial dural-based Ewing’s sarcoma in a pregnant female: A case report

Ewing's sarcoma (ES) is a rare and highly malignant tumor characterized by small round blue cells, typically originating from bones and soft tissues in children and young adults, although it is rarely found as a primary intracranial lesion. We presented a case of a 26-year-old primigravida presenting with headache, vomiting, blurred vision, papilledema, and abducens nerve palsy. Magnetic resonance imaging (MRI) scan revealed a large extra-axial cystic-solid mass located in the occipitoparietal region, which resembled an atypical meningioma. Surgical excision was carried out, and histopathological examination, along with immunohistochemistry, confirmed the diagnosis of ES, which tested positive for CD99, NKX2.2, and EWSR1 gene. Whole-body imaging excluded any extracranial primary sites or metastasis, confirming the presence of a solitary primary intracranial ES. After the operation, the patient was treated with vincristine-doxorubicin-cyclophosphamide and ifosfamide-etoposide chemotherapy, which resulted in a partial radiological response. This case illustrates the diagnostic difficulties associated with intracranial ES due to its radiological similarity to meningioma and stresses the importance of immunohistochemical and genetic analysis for a conclusive diagnosis. Early multimodal management is vital for improving survival in such rare cases.