Meningioangiomatosis: A review of the variable manifestations and complex pathophysiology

Area of Science:

• Neuropathology
• Neuro-oncology
• Vascular Neurology

Background:

• Meningioangiomatosis (MA) is a rare, heterogeneous disease characterized by meningovascular proliferation.
• It primarily affects the leptomeninges and cerebral cortex but can involve other brain regions and occur in pediatric or adult populations.
• MA can be sporadic or associated with neurofibromatosis.

Purpose of the Study:

• To review the diverse clinical, radiological, and pathological manifestations of meningioangiomatosis.
• To discuss associated neurological diseases, tumor types, and conditions.
• To summarize current treatment approaches and proposed pathogenetic theories for MA.

Main Methods:

• Literature review of meningioangiomatosis (MA).
• Analysis of MA variants including neurofibromatosis-associated, multifocal, cystic, and those associated with other pathologies.
• Review of treatment strategies and pathogenetic hypotheses.

Main Results:

• MA presents with highly variable clinical symptoms, radiological findings, and pathological features.
• Manifestations include associations with meningioma, other brain tumors, focal cortical dysplasia, neurodegeneration, and post-radiation changes.
• Pathogenesis remains unclear, with theories suggesting developmental, dysplastic, hamartomatous, or reactive origins.

Conclusions:

• Physician awareness of MA's complexity and variability is crucial for timely diagnosis.
• Further research is needed to elucidate MA pathogenesis and improve patient outcomes.
• MA represents a spectrum of meningovascular proliferation requiring comprehensive understanding and management.