Childhood meningiomas associated with meningioangiomatosis: report of five cases and literature review.

Meningioangiomatosis is a unique, rare hamartomatous lesion. Meningiomas arising in the background of meningioangiomatosis are rare conditions which pathologically and radiologically mimic invasive meningiomas, but have a benign clinical course in children and young adults. In this study, five such cases are reported. To our knowledge, this is the largest reported collection of meningiomas associated with meningioangiomatosis. Less immunoreactivity for progesterone receptor and high Ki-67 labelling index are generally known to be associated with invasive meningiomas. However, high expression of progesterone receptor and low Ki-67 labelling index in the present cases supports the idea that brain invasion is not an indicator of malignancy but an independent finding associated with meningiomas which have arisen from meningioangiomatosis. We emphasize the good prognosis of such tumours and discuss pathogenesis of meningiomas with meningioangiomatosis.