Meningioangiomatosis

Abstract

A 58-year-old man presented with a 6-month history of headaches, progressive left homonymous hemianopia, and right-sided paresthesias. MRI revealed bilateral parietal and occipital enhancing lesions (figure 1, A-C) suggestive of cerebritis or posterior reversible encephalopathy syndrome due to history of hypertension. Worsening vision prompted neuroimaging 18 months later, showing contrast-enhancing cortical lesions in the posterior frontal region; fluid-attenuated inversion recovery abnormalities in the white matter (figure 1, D-F) suggested vasogenic edema and mass effect. Occipital lobe biopsy revealed cortical mesenchymal capillary proliferation typical of meningioangiomatosis (figure 2), a rare benign progressive disorder characterized by cortical vascular proliferation with associated perivascular mesenchymal cell proliferation.1 Seizures are the presenting feature in 85% of sporadic cases; there have been no reports with permanent focal dysfunction 2 as in this instance.

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

“…The limited number of MA-M cases may account for such a difference. MA usually presents with a male predisposition [69], consistent with the literature [4], and male predominance was more obvious in MA-M (unlike conventional meningioma) than in pure MA. This finding suggests that hormones may play an important role in the pathogenesis of MA-M. Wiebe et al suggested that the temporal lobe was the most frequently involved in MA [1], while the frontal lobe seemed to be more affected, based on our analysis.…”

“…Pure MA can even present as meningioma-like dural attachment and homogeneous contrast enhancement [21,28]. In a few reported cases [1,17,32,45,69,73,80], including case 8 and case 9 in our series, pure MA has exhibited a unique presentation of gyriform alteration or gyriform enhancement (Fig. 1).…”

“…A single lesion was the predominant pattern of sporadic MA, with only six patients presenting with bilateral or multifocal lesions [24,39,60,66,69]. The frontal and temporal lobes were affected in 48 (32.2 %) and 52 patients (34.9 %), respectively.…”

Progressive diffuse meningioangiomatosis: Response to bevacizumab treatment

“…2 Bilateral occipital cortical involvement with associated progressive cortical blindness, similar to our case, has been reported only once previously. 5 The only known treatment for meningioangiomatosis is surgical resection; however, in this unique case, surgical resection was not feasible because of the extensive, diffuse nature of the disease. 2 Since one of the primary histopathologic features of meningioangiomatosis is meningovascular proliferation, we hypothesized that antiangiogenic therapy, such as bevacizumab, a recombinant humanized monoclonal antibody that blocks angiogenesis by inhibiting vascular endothelial growth factor A, might be beneficial.…”

“…4 Sporadic cases are typically solitary and symptomatic, while NF2-associated meningioangiomatosis is often multifocal and asymptomatic. 5 The primary histopathologic features are leptomeningeal and perivascular mesenchymal cell proliferation and the presence of dysplastic neurons. Immunohistochemistry is of limited diagnostic value.…”