Primary, Dural-based, Ewing sarcoma in a pediatric patient: presentation of a rare tumor entity with literature review.

Abstract

Primary Ewing's sarcoma originating from the calvaria bone and/or underlying Dural involvement has been reported relatively rarely in the literature. Those originating from the dura and invading the bone above it in both directions, both towards the brain parenchyma and via the dura, are even rarer. CASE DESCRIPTION: We present a case of a 14-year-old girl with no known focal neurological deficit who presented with the complaint of vertigo for only 2months. In neuroradiological examination, the left frontoparietal region of the brain showed the presence of a tumor originating from the dura, invading both bone and brain parenchyma. No other tumor location was discovered after radiological examination. Since the patient had a shift in the brain and progressive loss of strength on the right side, the patient was taken to surgery for tumor excision. The frozen result sent per-operatively was consistent with a round blue cell tumor. Adjuvant chemotherapy treatment was given to the patient after the definitive pathology report was compatible with Ewing's sarcoma. CONCLUSION: The patient had an uneventful neurological recovery without permanent neurological deficit. When the patient was kept under close clinical and radiological surveillance 1year after the operation, no recurrence of the disease was observed. Bone marrow biopsy results and pet computerized tomography results confirmed the case of primary intracerebral Ewing sarcoma. This case illustrates an extremely rare location of primary Ewing's sarcoma with a set of clinical signs and symptoms extremely rare for this location of this rare disease entity.