Abstract
BackgroundEwing’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 presentationWe 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.ConclusionEwing’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.
Highlights
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
Multifocal intracranial extradural Ewing’s sarcoma has been described in the medical literature [4]. It can be misdiagnosed as central primitive neuroectodermal tumor, e.g., medulloblastoma, pinealoblastoma, or
In more than 97% of the cases, central nervous system (CNS)-EES have the expression of MIC-2 gene product, CD99, on their surface which can be detected by the monoclonal antibodies O13 and HBA71 [9]
Summary
This case is about a young patient, who presented with acute neurological symptoms, suggesting raised ICP or CNS infection. Initial neuroimaging revealed a biconvex intracranial extradural lesion that was eventually diagnosed as extraosseous intracranial Ewing’s sarcoma. This presentation is exceedingly rare, yet in the right context, Ewing’s sarcoma can be considered as one of the differential diagnoses of Epidural brain lesion
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