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Primary, Dural-Based, Ewing Sarcoma Manifesting with Seizure Activity: Presentation of a Rare Tumor Entity with Literature Review

Journal

WORLD NEUROSURGERY
Volume 129, Issue -, Pages 216-220

Publisher

ELSEVIER SCIENCE INC
DOI: 10.1016/j.wneu.2019.06.036

Keywords

Bone invasion; Ewing sarcoma; Intracranial-extracranial extension

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BACKGROUND: Primary Ewing sarcoma arising from the calvarial bone and/or underlying dura matter are relatively rarely reported in the literature. Even rarer are considered to be those that originate from the dura and proliferate in both directions, toward the brain parenchyma and through the dura invading the overlying bone. CASE DESCRIPTION: We report the case of a girl aged 11 years who presented with an ictus of generalized tonic-clonic seizures, without known focal neurologic deficit. Neuroradiologic work-up verified the existence of a tumor in the right fronto-parietal area of the brain, invading both the bone and brain parenchyma and emanating from the dura. No other tumor location was discovered after radiologic work-up, and gross total resection was undertaken followed by adjuvant radiation therapy and chemotherapy. CONCLUSIONS: The patient had an uneventful neurologic recovery without persistent neurologic deficit. One year after the operation, with the patient under close clinical and radiologic surveillance, no recurrence of the disease was detected. This case illustrates an extremely uncommon location of primary Ewing sarcoma, along with a constellation of clinical signs and symptoms that are fairly uncommon for this location of this rare disease entity.

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