Decoding Meningioangiomatosis: A Century of Insights into Molecular Mechanisms, Clinical Dilemmas, and Therapeutic Innovations.

Abstract

Meningioangiomatosis (MA) is a rare, enigmatic disorder straddling the boundary between developmental malformation and neoplastic process, characterized by meningothelial proliferation and vascular changes within the cerebral cortex. Despite its recognition over a century ago, MA remains diagnostically and therapeutically challenging due to its heterogeneous clinical presentations, overlapping radiological features with epileptogenic pathologies, and unresolved debates about its pathogenesis. This comprehensive review synthesizes a century of evidence to address critical gaps in the literature, including the molecular underpinnings of neurofibromatosis type 2-driven sporadic and syndromic MA, the diagnostic dilemmas posed by its histopathological mimicry of meningiomas and cortical dysplasia, and the absence of consensus on management strategies for asymptomatic or refractory epileptic cases. We present a visually reinforced, practical guide integrating advanced imaging hallmarks, immunohistochemical profiles, and surgical nuances-highlighting en bloc resection as the gold standard for seizure control (70-90% Engel Class I outcomes) while challenging the speculative role of radiotherapy given MA's low proliferative index. Controversies such as the hamartoma-neoplasm dichotomy, the inflammatory component's significance, and the potential of emerging therapies (e.g., mTOR inhibitors, bevacizumab) are critically evaluated. By proposing a diagnostic algorithm and management framework grounded in multimodal evidence, this review not only consolidates existing knowledge but also charts a roadmap for future research-emphasizing international registries, molecular profiling, and patient-centered outcomes. As the definitive resource for neurologists, neurosurgeons, and pathologists, this work bridges fundamental science with clinical pragmatism, aiming to transform MA from a diagnostic curiosity into a precisely managed entity.