Intracranial neurenteric cyst mimicking an ependymoma: imaging features, pathologic correlation and review of literature

Abstract We present a case of a 57-year-old female with four-months of diplopia and vertigo. MRI revealed a mixed cystic and solid partially enhancing lesion of the 4th ventricle, foramen of Luschka and cerebellopontine angle. Preoperative differential diagnosis favored ependymoma. Biopsy revealed a neurenteric cyst, a benign developmental lesion that rarely occurs intracranially. This case highlights several atypical manifestations of intracranial neurenteric cyst, with regions of histologically benign solid enhancement, multicompartmental extra-axial location mimicking an ependymoma, and rapid recurrence without evidence of underlying malignancy.