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Year : 2012  |  Volume : 55  |  Issue : 1  |  Page : 117-118
Meningioangiomatosis: A report on a rare case, masquerading as schwannoma

1 Department of Neuropathology, National Institute of Mental Health and Allied Neurosciences, SSSIHMS, Bangalore, Karnataka, India
2 Department of Pathology and Transfusion Medicine, SSSIHMS, Bangalore, Karnataka, India
3 Department of Radiology, SSSIHMS, Bangalore, Karnataka, India
4 Department of Neurosciences, SSSIHMS, Bangalore, Karnataka, India

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Date of Web Publication11-Apr-2012

How to cite this article:
Yasha T C, Ghosal N, Singh S S, Hegde A S. Meningioangiomatosis: A report on a rare case, masquerading as schwannoma. Indian J Pathol Microbiol 2012;55:117-8

How to cite this URL:
Yasha T C, Ghosal N, Singh S S, Hegde A S. Meningioangiomatosis: A report on a rare case, masquerading as schwannoma. Indian J Pathol Microbiol [serial online] 2012 [cited 2022 Jun 28];55:117-8. Available from: https://www.ijpmonline.org/text.asp?2012/55/1/117/94881

A 19-year-old male presented with five years history of gradually progressive headache and on and off generalized tonic clonic seizures. There was no family history of neurofibromatosis or stigmata of NF2. Computed tomography (CT) scan [Figure 1]a showed well defined globular hyper attenuated, peripherally calcified lesion measuring 3.5 × 2 ×2.3 cm in left medial temporal lobe with extension into anterior cranial fossa. Lesion appeared iso to mildly hypointense on T1 weighted images (WI), intensely hypointense on T2W and FLAIR [Figure 1]b magnetic resonance (MR) images and arise in the extra-axial compartment with invasion into adjacent brain parenchyma giving fluffy margin [Figure 2] in posterolateral aspect with streaks of linear hypointensity invaginating left orbital gyri [Figure 1]c. Post gadolinium T1WI showed inhomogeneous contrast enhancement with dural enhancement [Figure 1]d. A left pterional craniotomy and subtotal excision of the tumor was performed and sent for histopathological examination. On light microscopy, the sections showed a lesion composed of spindle shaped cells arranged in fascicles with focal nuclear palisading and presence of psamomma bodies [Figure 2]a-c. Cells appeared to emerge from the perivascular location in the meningeal surface and infiltrate cortex. Occasional whorl formation with prominent perivascular accentuation of the spindle cells [Figure 2]d in the intracortical region was noted with focal marked hyalinization. Few foci showed solid sheets of spindle cells with serpentine nuclei, wispy cytoplasmic borders without intervening cortex, resembling schwanomma [Figure 2] e-f. Despite cellularity, the proliferating cells in all cases had bland cytological features. There was sprinkling of lymphocytes with dysmorphic neurons in adjacent grey mater. Reticulin stain was confined around the blood vessels. On immunostaining, cells expressed vimentin uniformly [Figure 3]a and were negative for epithelial membrane antigen (EMA) and glial fibrillary acidic protein (GFAP) [Figure 3]b,e-f. Focal strong positivity for S-100 protein was noted [Figure 3]c-d. On overall histopathological findings, a diagnosis of meningioangiomatosis, left medial temporal lobe was given. Meningioangiomatosis (MA), first described in 1915, was named as "meningioangiomatosis" in 1937 by Worster-Drought et al. [1] Initially described in association with (NF-2), it is now known to occur predominantly sporadically, with more than 100 such cases described in literature. [2] The exact pathogenesis of MA is unknown; however, the possible theories are: A hamartomatous proliferation of the leptomeninges; direct invasion of the brain parenchyma by a leptomeningeal meningioma; and a vascular malformation inducing perivascular meningothelial proliferation of cells from vessel walls or from pluripotent arachnoid cap cells in Virchow-Robin spaces. [3] Sporadic MA are generally seen in younger age with mean of 21 years and reported frequently in males. More than 90% of MA is located in the cortex with temporal lobe being favorite location. Unusual locations like third ventricle and pulvinar-cerebral peduncle in those with NF; corpus callosum, trigeminal ganglion and medulla intra-axially in sporadic cases have been documented. [4] On imaging, plain CT characteristically show well defined calcific lesion. The lesion may be seen creeping into the brain parenchyma with areas of gliosis. MA may mimic other pathological processes on MRI, including meningioma, acute hemorrhage, calcified arteriovenous malformation, and encephalomalacia. On histopathology, MA has been classified as predominantly cellular and predominantly vascular. Our case was a predominantly cellular variant. The cells are consistently vimentin positive and often EMA negative. GFAP, S-100, and neuronal specific enolase show inconsistent staining and factor VIII is not expressed by the lesional cells. Our case showed focal strong positivity for S-100 protein in the spindle cells in cellular areas and intervening cortical tissue but not in perivascular cells. Ishihara et al. [5] reported case of intracerebral schwannoma in a 5-year-old boy showing distinctive plexiform growth pattern, and small aggregates of Schwann cells spread extensively into the surrounding brain tissue along perivascular spaces adjacent to the tumor nodule resembling meningioangiomatosis. There was diffuse and strong immunopositivity for S-100 protein in their case. Wiebe et al. [4] in a comprehensive study of MA found that S-100 immunopositivity was seen only in two cases; this positivity was seen only in the blood vessels and glial cells and not in the spindle cells unlike our case wherein focal strong immunopositivity was seen in the spindle cells.
Figure 1: (a) CT showing peripherally calcific lesion in left medial temporal lobe extending into anterior cranial fossa. (b) Axial FLAIR MRI showing intensely hypointense lesion with illdefined fluffy margin (arrow) in the posterolateral aspect. (c) Coronal T2WI showing linear streaks of hypointensity in olfactory sulcus and orbital gyri (curve arrow). (d) Post gadolinium enhanced T1WI showing inhomogeneous enhancement (thick arrow) with dural enhancement (thin arrow)

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Figure 2: Paraffin section of meningioangiomatosis showing (a,b) perivascular arrangement of spindle cells (curved arrow) with intervening cortical tissue, sprinkling of lymphocytes, (c) psamomma bodies and (d) serpentine nuclei at few foci (straight arrow). (e) Cellular areas showing spindle cells with no intervening cortical parenchyma resembling nerve sheath tumor. (f) High power view of the same. [Hematoxylin and Eosin (a, b, c, e)× 100; (d, f)× 400]

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Figure 3: On immunohistochemistry the cells are (a) vimentin positive, (b) GFAP negative (c) focally S-100 protein positive, (d) S-100 negativity in the peivascular cells, and (e, f) EMA negativity in both the cellular and perivascular regions. [Avidin Biotin Complex Immunoperoxidase: (a)× 100; (b-f)× 400]

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To conclude, we present a rare case of sporadic MA in a young male which showed focal cellular morphology like schwannoma with S-100 positivity resembling intracortical schwannoma. As these lesions are distinct clinicopathological entity and hamartomatous in nature, it is essential to make an accurate diagnosis to avoid unnecessary aggressive treatment.

   References Top

1.Worster-Drought C, Carnegie-Dickson WE, McMenemey WH. Multiple meningeal and perineural tumours with analogous changes in the glia and ependyma (neurofibroblastomatosis): With report of two cases. Brain 1937;60:85-117.  Back to cited text no. 1
2.Omeis I, Hillard VH, Braun A, Benzil DL, Murali R, Harter DH. Meningioangiomatosis associated with neurofibromatosis: Report of 2 cases in a single family and review of the literature. Surg Neurol 2006;65:595-603.  Back to cited text no. 2
3.Kissil JL, Johnson KC, Eckman MS, Jacks T. Merlin phosphorylation by p21 activated kinase 2 and effects of phosphorylation on merlin localization. J Biol Chem 2002;277:10394-9.  Back to cited text no. 3
4.Wiebe S, Munoz DG, Smith S, Lee DH. Meningioangiomatosis: A comprehensive analysis of clinical and laboratory features. Brain 1999;122:709-26.  Back to cited text no. 4
5.Ishihara M, Miyagawa-Hayashino A, Nakashima Y, Haga H, Takahashi JA, Manabe T. Intracerebral schwannoma in a child with infiltration along perivascular spaces resembling meningioangiomatosis. Pathol Int 2009;59:583-7.  Back to cited text no. 5

Correspondence Address:
Nandita Ghosal
Department of Pathology and Transfusion Medicine, Sri Sathya Sai Institute of Higher Medical Sciences, EPIP Area, Whitefield, Bangalore - 560 066
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.94881

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