4.3 Article

Molecular Alterations in Meningioangiomatosis Causing Epilepsy

Journal

JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
Volume 80, Issue 11, Pages 1043-1051

Publisher

OXFORD UNIV PRESS INC
DOI: 10.1093/jnen/nlab095

Keywords

Epilepsy; Meningioma; Meningioangiomatosis; Molecular alterations; NF2

Funding

  1. National Cancer Institute of the National Institutes of Health [K08CA241651]

Ask authors/readers for more resources

The study evaluated molecular alterations in Meningioangiomatosis (MA) and found that some MAs may arise from meningiomas rather than transform into meningiomas. Although known oncogenic drivers were not detected in the 3 cases of pure MA, MA-meningiomas exhibit a neoplastic nature.
Meningioangiomatosis (MA) is a rare process at the intersection of cerebral developmental and neoplastic disorders that often results in epilepsy. We evaluated molecular alterations in MA to characterize its biology and pathogenesis. We searched a comprehensive institutional database for patients with MA treated between 2004 and 2019. Demographic, clinical, surgical, and radiographical data were collected. MA and associated meningioma tissues were evaluated using a next-generation sequencing assay interrogating 1425 cancer-related genes. We studied 5 cases: 3 with MA and 2 with MA associated with a meningioma. Of the MAs associated with a meningioma, 1 had deletions in the NF2 gene in both the MA and the meningioma components, whereas the other had an NF2 deletion in only the MA component. Additional mutations were identified in the MA components, suggesting that MA arises from the meningioma rather than the meningioma resulting from a transformation of the MA. The 3 cases of pure MA showed variants of unknown significance with no alterations in known oncogenic drivers. Our findings provide a starting point to a better understanding of the pathogenesis of this rare lesion. Our study indicates that MA-meningiomas have a neoplastic nature that differs from the hamartomatous/developmental nature of pure MA.

Authors

I am an author on this paper
Click your name to claim this paper and add it to your profile.

Reviews

Primary Rating

4.3
Not enough ratings

Secondary Ratings

Novelty
-
Significance
-
Scientific rigor
-
Rate this paper

Recommended

No Data Available
No Data Available