Meningioangiomatosis (MA) is a rare, benign neoplastic disorder involving the cortex and leptomeninges, the sporadic form, commonly presents as refractory localization-related epilepsy, but could be asymptomatic especially in older patients. The imaging features may be entirely non-specific. Magnetic Resonance Imaging (MRI) erroneously suggests meningioma, lowgrade tumour or vascular malformations.
The pathological findings are characterised by proliferation of meningothelial cells and leptomeningeal vessels and calcifications within the mass. Macroscopically there is dense thickening in the underlying cortex, often in a sharply defined area.
In this article we report 3 cases of MA, neither of whom had a familiary history or stigmata of Neurofibromatosis (NF). We discuss and place particular emphasis on the clinical presentation and diagnosis imaging, as well as on the outcome. We also review the literature concerning about the aetiology, pathology findings and imaging features of MA.
La meningioangiomatosis (MA) es un proceso neoplásico benigno raro, que envuelve el córtex y leptomeninges, en su forma esporádica comúnmente se presenta con epilepsia refractaria, en relación a su localización, pero puede ser asintomática, especialmente en adultos mayores. Las características de imagen pueden ser totalmente inespecíficas. Las imágenes de resonancia magnética erróneamente sugieren la presencia de un meningioma, tumores de bajo grado, o malformaciones vasculares.
Los hallazgos patológicos se caracterizan por la proliferación de células meningoteliales, vasos leptomeningeos y calcificaciones dentro del tumor. Macroscópicamente se observa un denso engrosamiento en el córtex adyacente, generalmente con un área claramente definida.
En este articulo, se describen 3 casos de MA, ninguno de ellos presentaba historia familiar o estigmas de neurofibromatosis (NF). Nosotros realizamos la discusión, haciendo particular énfasis en la presentación clínica y diagnóstico por imágenes, así como en el pronóstico.
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