Clear cell meningioma of the fourth ventricle

Clear cell meningioma (CCM) has been identified and included in the World Health Organization classification of CNS tumors recently. CCMs are histologically characterized by sheets of polygonal cells with clear cytoplasm, which is the expression of high glycogen concentration. Compared with other variants of meningiomas, CCMs occur in younger patients and usually are located in the spinal canal and posterior fossa, the last ones mainly in the cerebellopontine angle. Some reports suggest that CCMs have high recurrence rate and potentially aggressive behavior. Poor outcome has been shown in intracranial and spinal tumor location, but indicators that predict outcome have not been established. The authors present two cases of intracranial CCMs, with excellent outcome in patients harboring tumor location (restricted to the fourth ventricle) and some clinical particular features (young age, gender, obesity, and moderate impairment of intellectual capacity). To the best of our knowledge, these are the two first reported cases of clear cell meningioma located primarily in the fourth ventricle.