Supratentorial Neurenteric Cysts: Systematic Literature Review and Case Report

Background Neurenteric cysts (NC) are uncommon congenital lesions with histopathologic properties derived from the gastrointestinal or respiratory tract. They are typically located in the intradural extramedullary compartment but rarely seen in the supratentorial region. The occurrence of supratentorial NCs (S-NC) presents an interesting quandary regarding their embryopathogenesis. Methods We present a case report and systematic literature review on S-NCs following PRISMA guidelines. Results A 57-year-old woman presented with a seizure and paresthesias of the face, hands, and feet. Magnetic resonance imaging showed a right temporo-occipital cystic lesion, which was managed with surgical resection. Histologically, the cyst was type A. The patient was without recurrence at 10 months. Including this case, 88 S-NCs have been reported in the literature. Common presenting symptoms are headaches and seizures. They were mostly treated with craniotomy, preferably with gross total resection, although subtotal resection may be necessary because of adhesions. Resection usually led to symptom improvement (61%). Malignant transformation was seen in 3%. Recurrence was seen in 17%, with a mean time to recurrence of 4.2 years, and was significantly more common after subtotal resection than gross total resection. Conclusions If surgically resected, the cyst wall specimen should be sent for pathology review, because of the potential risk for malignancy. If conservatively managed, serial imaging is warranted to track for changes that may indicate transformation. The embryopathogenesis of these rare congenital lesions remains incompletely understood, but the most comprehensive theory involves enteric cell migration to the neuroectoderm during embryogenesis.