Cranio-Cervical Junction Meningiomas

Meningiomas arising from the cranio-cervical junction (CCJ) are infrequent, benign lesions. Incidence of WHO grade 2 and 3 is significantly lower compared to convexity meningiomas. Clinical presentation may vary from a subtle neck pain to quadriparesis in the most severe cases, up to respiratory arrest. Diagnosis is made using contrast-enhanced MRI, but CT scan, MR angiogram and digital subtraction angiography may help in defining the surgical strategy. Endovascular procedures are sometimes used for balloon occlusion tests when one of the vertebral arteries is encased by the tumor. The most used classification, by Bruneau and George, divides CCJ meningiomas based on the location (anterior, lateral and posterior, intra- or extra-dural) and on the position relative to the vertebral artery. Since they grow in close proximity to critical neurovascular structures, surgical access is challenging. The preferred routes are the far-lateral and the posterior median suboccipital. Intraoperative monitoring is of utmost importance to preserve neurological function and quality of life. Recurrence rates are generally low; therefore, surgery should be more oriented to clinical preservation than to radical excision. Gamma Knife Radiosurgery is an effective treatment for selected patients.