We reviewed 36 patients with endodermal cysts occurring at the craniocervical junction. They were aged between 3 and 66 years. Headache, motor weakness, and neck pain were commonly observed symptoms. Radiographically, T1-weighted magnetic resonance imaging of the tumours demonstrated a hypointense, isointense, or hyperintense signal according to the cystic content. In most cases, the cyst walls did not enhance after gadolinium administration. Histologically, the cysts were found to be lined by a single layer of epithelium. Histochemical and immunohistochemical studies showed that almost all were reactive to periodic acid schiff stain, epithelial membrane antigen, and carcino-embryonic antigen, but negative to glial fibrillary acidic protein. Mainly, the suboccipital approach with or without a laminectomy, or the trans-oral approach were selected for surgical excision of these tumours. In 17 of the 36 patients, total or gross total resections were performed, and subtotal resections were achieved in sixteen. Three patients developed recurrences.
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