An Unusual Case of Large Posterior Fossa Neurenteric Cyst Involving Bilateral Cerebellopontine Angle Cisterns: Report of a Rare Case and Review of Literature

Abstract

SummaryBackgroundIntracranial neurenteric cysts are rare cystic masses of endodermal origin lined with mucin producing low columnar or cuboidal epithelium. Approximately 141 cases have been reported so far. Most of the posterior fossa neurenteric cysts are typically small, located anteriorly to the brainstem in the midline or in the cerebellopontine angle cistern area.Case ReportWe present a rare, histologically proven case of a large lobulated intracranial neurenteric cyst measuring 4.2 centimeters in the maximal transverse dimension and involving bilateral cerebellopontine angle cisterns. We also present a review of the literature on this uncommon finding.ConclusionsImaging features of neurenteric cyst are non-specific and it should be considered in the differential diagnosis for any intracranial extraaxial cystic lesion.

Supratentorial endodermal cysts - Report of two cases

“…EC originating in the CNS is most commonly located intraspinally, generally ventral to the spinal cord and is usually seen in the lower cervical and upper thoracic regions. [ 3 4 5 6 ] In the intracranial compartment, these are usually located in the posterior fossa, explained by the rostral closure of the notochord forming the clivus. Graziani et al .…”

“…EC is nonenhancing, but rare cases of rim enhancement and sometimes solid enhancement occur. [ 3 ] The differential diagnosis for intracranial EC on imaging include epidermoid/dermoid cysts, parasitic cysts, true neuroepithelial cysts, and arachnoid cysts. [ 6 ] Diagnosis of an EC is confirmed on histology.…”

“…The ideal treatment would be complete surgical excision, along with the cyst wall, with follow-up serial imaging for potential complications such as recurrence, dissemination, and malignant transformation. [ 3 6 ] However, the rarity of this location precludes a general consensus on the most appropriate line of management. We believe that the cysts which are symptomatic ought to be excised, the others can be closely observed.…”