Meningioangiomatosis: clinical-radiological features and surgical outcome

Abstract

SummaryMeningioangiomatosis (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.KEY WORDS: Brain tumor. Histopathology. Meningioangiomatosis. Magnetic resonance imaging. Seizure. Meningioangiomatosis: características clínico-radioló-gicas y resultados quirúrgicos ResumenLa 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.

Sporadic meningioangiomatosis with and without meningioma: analysis of clinical differences and risk factors for poor seizure outcomes

“…This suggests that, as a degenerative change, calcification on CT may be one of the most helpful features favoring the diagnosis of MA [39,83]. In our analysis, we found the majority (73.2 %) of sporadic MA cases present hyperintensity rather than a mixed signal on T2WI as previously described [1,24,83]. MA is believed to be mainly confined to the cerebral cortex; the subcortical white matter is rarely involved [1,45,74].…”

“…Half of the sporadic MA cases (51.6 %) showed calcifying changes on CT, corresponding to the 55 % reported in the literature [1]. This suggests that, as a degenerative change, calcification on CT may be one of the most helpful features favoring the diagnosis of MA [39,83]. In our analysis, we found the majority (73.2 %) of sporadic MA cases present hyperintensity rather than a mixed signal on T2WI as previously described [1,24,83].…”

Clinical Reasoning: Unusual headaches

“…3 Some studies suggest that meningioangiomatosis has predilection for male gender 3 but this is not consistent in other studies. 8 The mean age of presentation is 21 years for patients without neurofibromatosis and 28 years for patients with associated neurofibromatosis. 3 The treatment for meningioangiomatosis is surgical resection and it is curative if a total resection is achieved.…”

“…3 The treatment for meningioangiomatosis is surgical resection and it is curative if a total resection is achieved. 8 Meningioangiomatosis represents about 3% of brain tumors associated with medically intractable epilepsy. 9 In a study of 7 patients with meningioangiomatosis and seizures who underwent tumor resection, seizure-free frequency was seen in 43%, improvement in seizure frequency was seen in 30%, and no improvement in 28%.…”

“…3 Radiologic characteristics suggestive of meningioangiomatosis include well-demarcated focal lesions that are isointense or hypointense in T1-weighted images, commonly surrounded by an area of increased intensity on T2-weighted images, presence of calcifications, and nonhomogenous contrast enhancement. 3,8 A presurgical diagnosis remains difficult because the diagnostic tools lack specificity.…”