Multiple neurenteric cysts in the posterior fossa and cervical spinal canal - Case report

46-year-old woman presented with multiple neurenteric cysts in the posterior fossa and spinal canal. Neuroimaging demonstrated neurenteric cysts in the interspace between the left cerebellar hemisphere and vermis, the lateral side of the right cerebellar hemisphere, and the ventral side of the spinal cord at the C-2 and C-4 levels. Total resection of the paravermian cyst and partial removal of the spinal cyst at the C-4 level were performed. Histological examination showed the cyst wall consisted of single or multiple layers of columnar epithelial cells with secretory granules, with mucin secretion verified by periodic acid-Schiff staining. Immunohistochemical staining showed the walls were positive for the cytokeratin, epithelial membrane antigen, and carcinoembryonic antigen, and negative for glial fibrillary acidic protein and S-100 protein. These findings confirmed the endodermal origin. The diagnosis was neurenteric cyst. The paravermian cyst disappeared, but the spinal cyst at the C-4 level recurred 8 months later. Reoperation became necessary 16 months later. The other two cysts also showed enlargement at 6 or 15 months. Total removal of neurenteric cyst is recommended if possible.