An unusual biconvex epidural lesion: acutely presenting extraosseous intracranial Ewing’s sarcoma

BACKGROUND: Ewing’s sarcoma family of tumors consists of small round cell neoplasms, inclusive of primitive neuroectodermal tumor (PNET), Askin’s tumor, and PNET of the bone. Extraosseous Ewing’s sarcoma occurs commonly at bones of lower extremities and paravertebral region of the spine. It rarely presents as a primary intracranial lesion. When intracranial, it can be misdiagnosed as central PNET (e.g., medulloblastoma, pinealoblastoma, or supratentorial PNET), other intracranial lesions, or even as an epidural hematoma. CASE PRESENTATION: We report the case of a 20-year-old patient who presented to the emergency department with complaints of drowsiness, headache, and fever for 1 day. On initial computed tomography (CT) scan of the brain, a right temporal biconvex epidural lesion involving squamous-temporal bone with periosteal reaction was noted. The patient underwent urgent craniotomy, and a tumor was found and excised. Biopsy report confirmed Ewing’s sarcoma. CONCLUSION: Ewing’s sarcoma is a rare intracranial malignancy with only a few cases reported in literature. In a young patient with a biconvex epidural lesion, in the absence of trauma or ongoing infection, the possibility of Ewing’s sarcoma should be considered as well.