Intraspinal Neuroendodermal Cyst (Neurenteric Cyst) An Unusual Case Report and Literature review

Intracranial cystic lesions represent a heterogeneous group of conditions, including infections, abscesses, developmental abnormalities such as cysts, and neoplasms. Neuroendodermal cysts (also known as neurenteric cysts) are rare benign lesions that originate from the endoderm during the third week of embryonic development.   Neuroendodermal cysts are associated with high morbidity and can significantly diminish patients’ quality of life. Proper treatment and adequate histopathological examination are fundamental to improving patient prognosis. A 19-year-old female presented with progressively worsening gait difficulty and low back pain. Magnetic resonance imaging (MRI) revealed a heterogeneous lesion suspicious for a neoplastic process. Based on the radiological findings, surgical excision of the lesion was performed, and the specimen was submitted for histopathological evaluation. Gross examination revealed an opened cystic lesion measuring 2 × 1.6 × 0.7 cm. The cyst had a smooth surface with focal soft yellow areas. Microscopic examination showed that the cyst wall was lined by stratified columnar to cuboidal epithelium, with focal ciliation and the presence of goblet cells. No cytological atypia or evidence of malignancy was identified. Immunohistochemical staining demonstrated that the epithelial cells were positive for EMA and negative for S100 and LCA. Based on these findings, a diagnosis of neuroendodermal cyst was established. Central nervous system diseases encompass a wide range of lesions that may present with similar clinical features; however, histopathological examination remains the gold standard for accurate diagnosis. In this study, we discuss neuroendodermal cysts and provide a review of the literature, with particular emphasis on their histopathological features and immunohistochemical profile.