Meningioma caused by intense cerebral meningioangiomatosis with neural dissemination and nerve permeation

Abstract

Meningioangiomatosis (MA) is a rare benign hamartomatous lesion within the leptomeninges and cerebral cortex. The association with meningioma is very rare. We present a 30-year-old man, with no previous history of NF2, who started with progressive headache, decreased visual acuity, which progressed to total amaurosis of the left eye. Radiological studies showed a homogenous lesion located in the left frontotemporal region originating from the anterior clinoid process. He underwent surgery and a reddish tumor with a hemorrhagic spongy appearance that corresponded to numerous partially fibrous blood vessels, with proliferation of elongated meningothelial cells that formed solid areas with the appearance of a meningothelial meningioma. It was positive for EMA, vimentin, and Factor VIII showed positivity in the perivascular proliferating cells and negative in the solid areas. With the reticulin stain we observed alterations in the wall of the thick and large vessels. This case is discussed since it presents as a cerebral mass formed by abnormal vessels with solid areas that corresponds to meningioma.