Histopathological study of five cases with sporadic meningioangiomatosis

Abstract

We report five cases of sporadic meningioangiomatosis, three males and two females, ranging in age from 12 to 36 years at diagnosis. The lesion was found incidentally by MRI after a head trauma in one case; the other four subjects had a seizure disorders, which improved following surgical resection of the cortical lesions. Grossly, the lesionectomy specimens were of a whitish color and firm consistency. Histological examination revealed that the lesions were confined to the cortex with focal involvement of the overlying leptomeninges, and revealed unifying features of meningioangiomatosis, such as proliferating microvessels with perivascular cuffs of spindle-cell proliferation within the cortex. Two cases had numerous calcifications; one was associated with a prominent fibrocalcifying component. Immunostaining results were variable among the cases. Only vimentin was consistently positive. Some of the spindle cells were weak positive for EMA in two cases. Immunoreactions with anti-CD34 detected within the cytoplasm of the spindle cells were observed in three of the five cases. The Ki-67 proliferation index of all the cases was very low, less than 0.1%. Neurofibrillary tangles were identified in only one of the five cases using the Bodian and immunostaining methods. These findings indicate that meningioangiomatosis lesions show a wide range of clinicopathological features, making diagnosis difficult. A histopathological spectrum and differential diagnoses were discussed with a review of the literature. Since this lesion is a distinct clinicopathological entity and hamartomatous in nature, it is important to make a correct diagnosis in order to avoid further aggressive treatment.

Meningioangiomatosis: clinical-radiological features and surgical outcome

“…Immunohistochemistry has limited diagnostic value as staining patterns vary between cases 12 . Only viementin, as a non-specific marker of the mesenchymal cell was consistently positive 27 . Nonetheless, inmmunohistochemistry may aid in the differentiation and exclusion of other disorders such as meningioma and schwannoma, because the latter shares common histological features with MA.…”

“…The commonest finding on computerised tomography (CT) scan is usually iso-to slightly hyperintense that show variable amount of calcification with little or no contrast enhancement but low-density lesions and normal CT images have also been reported 11,22,27 . MRI are confined to the cortex and consist of iso or hypointensity on T1-weighter images and heterogeneous cortical mass surrounded by an area of increased intensity on T2-weighter images, probably due to oedema or gliosis 6,18,25 .…”

Meningioangiomatosis in a 5-Year-Old Boy Presenting with Intractable Seizures

“…In prototypical cases, the two entities are distinct lesions, since the meningioma is an extra-axial meningothelial mass, while the meningioangiomatosis is a plaque-like cortical lesion of more fibroblastic nature. In rare coexisting cases, the lesion has intermediate histological features [8,9]. Although our case showed a meningothelial predominance, the cortical localization, the focal fibroblastic appearance and the obvious vascular component favored meningioangiomatosis.…”

“…It is usually a single cortical lesion; however, multifocal non-cortical cases have been reported [6,7]. Sporadic meningioangiomatosis is usually a single lesion occurring in young adults or children, who present with seizures or headache [6,8]. In contrast, NF2-associated cases are usually multifocal, generally asymptomatic and discovered postmortem [6].…”

“…Although heterogeneous diffuse expression of epithelial membrane antigen was observed in the spindle of our case, immunostaining results are variable in meningioangiomatosis. According to previous studies, the spindle cell component shows constant expression of vimentin and variable detection of epithelial membrane antigen and CD34 [8]. The Ki-67 proliferation index of all the cases was very low, less than 0.1% [8].…”