Blackwell Publishing AsiaMelbourne, AustraliaNEUNeuropathology0919-65442006 Blackwell Publishing Asia Pty LtdJune 2006263249256MiscellaneousSporadic meningioangiomatosisY Wang
et al.

Neuropathology 2006; 26, 249–256

doi:10.1111/j.1440-1789.2006.00668.x

E a s t Asi a Fr iend s hip Wo r ks hop

Histopathological study of five cases with sporadic
meningioangiomatosis
Yin Wang,1 Xiang Gao,2 Zhen-Wei Yao,3 Hong Chen,1 Jing-Jing Zhu,1 Shan-Xiang Wang,1
Ming-Shi Gao,1 Liang-Fu Zhou2 and Fu-Lin Zhang1
1

Department of Neuropathology, Institute of Neurology, 2Department of Neurosurgery, and 3Department of Radiology,
Huashan Hospital, Fudan University, Shanghai, China

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.

Correspondence: Yin Wang, MD, Department of Neuropathology,
Institute of Neurology, Huashan Hospital, Fudan University, 12
Wu Lu Mu Qi Zhong Road, Shanghai 200040, China. Email:
yinwang88@hotmail.com
Received 25 July 2005; revised and accepted 31 August 2005.

© 2006 Japanese Society of Neuropathology

Key words: hamartomatous lesion, histopathology, meningioangiomatosis, neurofibrillary tangles, seizures.

INTRODUCTION
Meningioangiomatosis (MA) is a rare, benign, meningiovascular hamartomatous condition affecting the cerebral
cortex and leptomeninges. Approximately 90 cases of MA
have been reported since the first description in 1915.1 The
majority of the cases are sporadic but the association of
this lesion with familial neurofibromatosis type 2 is well
known.2–8 MA usually appears in young patients. Focal seizures and/or headaches are the most common presenting
symptoms. A few cases with neurofibromatosis may be
asymptomatic, and have been found incidentally after a
head trauma or at autopsy. Histopathologically, the condition is characterized by cortical meningiovascular proliferation, perivascular spindle-cell proliferation through
Virchow-Robin spaces, fibrosis, hyalinization, calcification
and even osseous metaplasia. The pathogenesis of MA is
still obscure. Previous reports have suggested that the
origin of MA lesions is most probably a hamartomatous
proliferation of meningothelial cells.9–11 In this study we
present the histopathological features of five cases without
stigmata of neurofibromatosis type 2. The histopathological spectrum and differential diagnoses are discussed with
a review of the literature.

MATERIALS AND METHODS
We reviewed the neuropathological records at our department to find all cases of MA that were recorded from 2000
to 2004. Five cases were identified, three patients are male
and two are female, ranging in age from 12 to 36, mean
21.6. One, whose lesion was found incidentally after a head
trauma, was asymptomatic. Four had histories of seizures.
No stigmata of neurofibromatosis type 2, including bilateral acoustic Schwannomas or masses of the spinal cord,

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Y Wang et al.

Table 1 Clinical summary of five cases with MA
Case

Age/
sex

Duration of
illness (years)

Clinical
presentation

Neurological
examination

Location

Clinical diagnosis

Surgical treatment

1
2
3
4
5

26/M
15/F
36/F
19/M
12/M

–
3
0.5
0.5
7

Asymptomatic
Refractory seizures
Seizures
Seizures
Refractory seizures

Normal
Normal
Normal
Normal
Normal

Right temporal lobe
Right occipital lobe
Left frontal lobe
Left parietal lobe
Left frontal lobe

Calcific lesion
AVM
Low grade glioma
CA
Low grade glioma

Gross total resection
Gross total resection
Gross total resection
Gross total resection
Gross total resection

AVM, arteriovenous malformation; CA, cavernous angioma.

were found using MRI or family history in any of the cases.
General physical and neurological examinations were
normal. Follow-up duration ranged from 10 to 48 months.
In one case (patient 1) follow-up was not possible after
surgery. Detailed clinical information is summarized in
Table 1.
The resected tissues were fixed in 10% neutral formalin
solution. Paraffin-embedded 6-µm thick sections were then
stained with HE, Klüver-Barrera (KB), reticulum fiber
and Bodian methods. Immunohistochemical examinations
were performed using the standard streptavidin technique
with appropriate positive and negative controls. The following antibodies were employed: epithelial membrane
antigen (EMA, M0613, DAKO, Glostrup, Denmark,
1:100), Vimentin (M0725, DAKO, 1:100), CD34 Class II
(M7165, DAKO, 1:50), S-100 protein (Z0311, DAKO,
1:100), Smooth Muscle Actin (SMA, M0851, DAKO,
1:200), Cytokeratin (M3515, DAKO, 1:100), Glial Fibrillary Acidic Protein (GFAP, M0761, DAKO, 1:100), Synaptophysin (A0010, DAKO, 1:200), Neurofilament Protein
(M0762, DAKO, 1:100) Ubiquitin (Z0458, DAKO, 1: 100),
Tau (A0024, DAKO, 1:100) and Ki-67 (M7240, DAKO,
1:50).

RESULTS
Clinical summary
Case 1, a 26-year-old man with an unremarkable medical
history, was admitted to our hospital in March 2000 due to
an incidental head trauma. Head computed tomography
(CT) scan revealed a well-demarcated calcific lesion in the
right temporal lobe with surrounding low density. Clinically, calcific lesion was suspected. Intraoperatively, the
leptomeninges and subarachnoid space overlying the right
temporal region contained a 2.5-cm spherical calcification
filling a sulcus and compressing the cortical surface. The
calcific lesion and surrounding infiltration were totally
removed. Unfortunately, after the operation its follow-up
was not possible.
Case 2 was a 15-year-old girl with a history of complex
partial seizures for 3 years who was admitted to our hospi-

tal in August 2000 as a result of progressively intractable
complex partial seizures with secondary generalization.
She also suffered from headache and vomiting. Head CT
scan revealed a low-density lesion in the right occipital
lobe. On MRI, the cortical lesion was hypointense on T1weighted images and showed low signal on T2-weighted
images with surrounding hyperintense lesion. Radiologically, arteriovenous malformation was suspected. The
lesion and adjacent cortex were resected. The postoperative course was uneventful and no neurological deficits
were noted. A 4-year follow-up did not reveal any recurrence of the lesion.
Case 3, a 36-year-old woman with an unremarkable
medical history, was admitted to our hospital in August
2001 due to generalized seizures, which had developed
6 months previously. Brain MRI revealed a left frontal
mass lesion whose signal was hypointense on T1-weighted
images and hyperintense on T2-weighted images, with
surrounding increased T2 signal. A low-grade glioma
was suspected. She underwent left frontal craniotomy
and lesionectomy. The lesion appeared gray, firm, and
well demarcated. Gross total removal of the lesion was
achieved. The postoperative course was uneventful and no
neurological deficits were noted. A 3-year follow-up did
not reveal any recurrence of the lesion.
Case 4 was that of a 19-year-old man with an unremarkable medical history who was admitted to our hospital in
June 2004 due to generalized seizures, which had developed 6 months previously. Brain CT revealed a 2.0-cm
well-demarcated ovoid mass without peritumoral edema in
the left superficial gyrus of the parietal lobe (Fig. 1). Clinically, cavernous angioma was suspected. Intraoperatively,
the mass was a well-demarcated, gray and stony, hard,
globular lesion. Gross total removal of the lesion was
achieved. During the 10-month follow-up, there was no
recurrence of seizure or tumor.
Case 5, a 12-year-old boy with progressively intractable
complex partial seizures since the age of 5, was admitted to
our hospital in July 2004 as a result of headache and vomiting. There was no history of perinatal problems, feverrelated seizures or developmental delay. A brain MRI
revealed a cystic lesion with increased cortical signal in the
© 2006 Japanese Society of Neuropathology

Sporadic meningioangiomatosis

251

Fig. 1 Axial CT scan showing a nodular, calcific mass in the
left parietal lobe.

Fig. 2 Sagittal MRI T1-weighted image showing a cystic
lesion with increased cortical signal in the left frontal lobe.

Table 2 The main histopathological findings of five cases
Case

1
2
3
4
5

Meningiovascular
proliferation

Perivascular cuffs
of spindle cells
proliferation

Perivascular
connective tissue
proliferation

Reticulum
fiberde
position

Calcification

+
+ (dominant)
+
+
+

+
+
+ (dominant)
+ (dominant)
+ (dominant, involving
the white matter)

+
+
+ (dominant)
+
+

+
+
+
+
+

+ (fibrocalcifying formation)
–
–
+
–

Nervous tissue
Gliosis NFT
+
+
+
+
+

+
–
–
–
–

NFT, neurofibrillary tangles.

left frontal lobe on T1-weighted image, interpreted as
low-grade glioma or cystic encephalomalacia (Fig. 2). The
patient underwent left frontal craniotomy and lesionectomy. Postoperatively, he continued to take anticonvulsant
medication and has remained seizure-free with no evidence of lesion recurrence for 10 months.

Histopathological findings
All five cases showed similar histopathological features.
The main histopathological findings are summarized in
Table 2.
In case 1, the lesionectomy specimen contained a 3.0-cm
calcification largely filling a sulcus in the leptomeninges.
© 2006 Japanese Society of Neuropathology

The adjacent cortex was of a whitish color and firm consistency. Microscopically, the calcified leptomeningeal mass
was composed of multilobulated amphophilic nodules with
a concentric arrangement of acellular lamellae, some were
centrally dense, hyalinized and resembled fibrocalcifying
component (Fig. 3A). The adjacent cortex showed rows
of proliferating oval to spindle cells with hyperchromatic
nuclei, psammoma bodies, and numerous slit-like capillaries. Neither mitosis nor necrosis was found. Histopathology confirmed the diagnosis of MA.
In case 2, the surgical specimen, measuring 1.0 cm in
diameter, was gray. Microscopically, the lesion was confined to the cortex with focal involvement of the overlying
leptomeninges. The adherent cortex showed prominent

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Y Wang et al.

Fig. 3 Light microscopic features of the MA: (A) case 1, fibrocalcifying nodule with spindle cells proliferation in the adjacent cortex (HE, ×40); (B) case 2, predominantly vascular case with numerous capillaries and venules in the cortex (HE, ×200); (C) case
3, highly cellular area showing fascicular pattern; there is complete effacement of cortical architecture (HE, ×100); (D) case 4, some
vessels of the cortex with thickened vascular wall and minimal cuffs of spindle-cell proliferation (arrows) (HE, ×100); (E) case 5,
cut sections revealed a pale, rubbery and gritty intracortical lesion with cystic formation; and (F) case 5, perivascular spindle-cell
proliferation presenting in the white matter (HE, ×100).

© 2006 Japanese Society of Neuropathology

Sporadic meningioangiomatosis
proliferation of microvessels, mainly capillaries and
venules (Fig. 3B). Cuffs of spindle cells surrounded them.
No calcification was noted. These findings were similar to
MA with a predominant vascular pattern according to the
classification of Wiebe et al. 12
In case 3, the lesionectomy specimen, measuring
3.5 × 2.5 × 1.2 cm, was of a whitish color and firm consistency. Microscopically, the most prominent feature of the
transcortical plaque was a complex of small-caliber blood
vessels lined by a single layer of endothelial cells and encircled by spindle cells in cuffs as well as parallel bundles
(Fig. 3C). Neither cellular atypia nor necrosis was found.
Some blood vessels had fibrosed or hyalinized vascular
walls. A dense reticulum and collagen fiber network was
aggregated around the bigger blood vessels. No calcification was noted. These findings suggested MA with a predominant cellular pattern according to the classification of
Wiebe et al. 12
In case 4, the lesionectomy specimen, measuring 1.2 cm
in diameter, had leptomeningeal calcific deposits and a cortical plaque. The main histopathological features of this
case were similar to case 3. It was characterized by a proliferation of leptomeningeal vessels and perivascular cuffs
of spindle cells within the cortex. The perivascular arrangement of spindle cells was more evident in the periphery
of the lesion. Some vessels were surrounded by two or
three layers of concentrically arranged spindle or oval cells
(Fig. 3D). Sparse lymphocytic infiltrations were present in
the leptomeninges and near some cortical vessels. Multiple
calcified depositions were found in the leptomeninges and
cerebral cortex. Abundant reticulum fiber with a pericellular distribution was observed. MA with a predominant cellular pattern was established in this patient.
In case 5, The lesionectomy specimen measured
4 × 3 × 2 cm. Cut sections revealed a pale, rubbery and
gritty intracortical lesion with cystic formation (Fig. 3E).
Microscopically, the lesion was composed of elongated
spindle cells with wavy nuclei, forming moderately- to
highly-cellular, focally palisading zones around fibrillar
areas in which cell nuclei were scarce. Although the compact areas were mainly localized to the cortex perivascular spindle-cell proliferation was also observed in the
white matter (Fig. 3F). No calcification was noted. The
appearance was that of MA with a predominant cellular
pattern.
On immunohistochemical analysis, the proliferating
spindle-cell population expressed vimentin uniformly.
Results for other markers varied: two cases (patients 4, 5)
showed focal EMA positivity and three (patients 1, 3, 4)
were focally positive for CD34. All were negative for
cytokeration, S-100 protein, SMA, GFAP and neurofilament. The Ki-67 proliferation index for all the five cases
was very low, less than 0.1%.
© 2006 Japanese Society of Neuropathology

253

Fig. 4 Tau-positive neurofibrillary tangles within cytoplasm
of neuron in the cortical lesion of case 1 (Tau-immunostain,
×400).

All the cases showed gliosis and neuronal loss with
residual neurons presenting with chronic non-specific
degenerative changes within the lesion areas and in the
neighboring cortex. Bodian staining and Ubiquitin,
Tau immunostaining demonstrated neurofibrillary tangles
(NFT) in the cortex adjacent to the lesion only in one case
(patient 1) (Fig. 4).

DISCUSSION
Meningioangiomatosis is treated by surgery and, after total
excision, the lesion does not regrow.13–16 Although we have
described five cases of sporadic MA, presurgical diagnosis
remains difficult because diagnostic tools lack specificity.
CT and MRI erroneously suggested low-grade glioma, vascular malformations and calcific lesion in our cases. CT and
MRI findings reported in MA are controversial; they
depend on the developmental histological status of the
lesion.17–19 Radiological appearances are mostly not specific for the diagnosis. On CT, MA is usually iso- to slightly
hyperintense with or without the features of calcification,
but low-density lesions and normal CT images have also
been reported.17,18 The MRI findings are variable. Partington and Nomura have reported iso-intensity with gray matter on T1 and hypointensity on T2-weighted images.20,21
Thus, a wide spectrum of imaging expressions of MA often
impedes the clinical diagnosis.
The histopathological features of our cases are summarized in Table 2. All our patients’ MA lesions were
confined to the cortex with variable involvement of the
overlying leptomeninges. Although all the cases had unifying features, such as cortical vascular proliferation and
perivascular cuffs of spindle-cell proliferation, each case
was unique. Wiebe et al. described the histopathological
spectrum of MA,12 which can be broadly classified into pre-

254
dominantly cellular and predominantly vascular types. The
predominant cellular pattern was to show moderate to high
cellularity, as in our present cases (patient 3, 4, and 5).
Varying architecture was noted, consisting of focal areas
of stroriform, rhythmic palisading and fascicular patterns.
All cellular cases had lesional cells that, in some areas,
appeared to emerge from a perivascular location and infiltrate the cortex. This occurred centrally within the lesions,
where cellularity was most dense. Peripherally, the perivascular relationship of the cells became evident. Moreover,
patient 5 showed the white matter involved. The predominantly vascular pattern, as in our patient 2, contained
thick-walled, hyalinized blood vessels and increases in
numbers with minimal perivascular cell proliferation.
Different forms of calcification are usually encountered in
MA. Our patient 1 showed the arrangement of concentric
lamination of calcific concretions. Similar changes were
also recorded in previous reports, termed fibro-osseous,
fibrocalcifying component or focal bony metaplasia.22–27 So,
the histopathological feature of patient 1 suggested MA
with a predominant fibrocalcifying formation. Our cases
indicated that the morphological changes of the MA
lesions were variable. Depending on the age of the lesion,
we consider that MA might be divided into three types,
predominantly cellular type, vascular type and fibrocalcifying type.
The histopathological diagnosis of MA depends on its
localization, size, and the chronicity of the lesion.11,14,22
Many cases showed proliferating perivascular cells infiltrating the cortex in association with marked cellularity
and reactive gliosis. Unless the pathologist is familiar with
the histopathological features of MA, these features may
lead to an erroneous diagnosis. The histopathological
differential diagnoses include meningiomas with cortical
invasion, vascular malformation and gliomas. Meningiomas exhibit a predominance of meningothelial cells and do
not have the pronounced vascularity seen in MA. An angiomatous meningioma contains numerous mature blood
vessels with meningothelial cells in varying proportions,
but neurons and glial cells are absent. An apparent meningothelial component may raise suspicion of an atypical or
anaplastic meningioma with cortical invasion, but a lack
of cellular atypia, mitotic activity, necrosis and cortical
destruction favor MA. Vascular malformation tends to
bleed, which is an extremely rare feature of MA since the
lesion is firm due to the presence of a variety of mesenchymal and neural elements.28,29 The presence of small-sized
vessels, their uniform distribution, lack of hemorrhage,
normal intervening tissue, associated spindle-cell proliferation and superficial location should preclude diagnosis of
vascular neoplasms. The elongated, spindly cells may be
mistaken for astrocytes. Furthermore, prominent endothelial proliferation might result in an erroneous diagnosis of

Y Wang et al.
a high-grade glioma. However, the absence of the typical
‘copper wire’ appearance of astrocytic fibrils and glomeruloid vessels and the presence of neurons within the lesion
should preclude that diagnosis. On the other hand, we
should pay great attention to recent reports concerning
MA coexisting with meningioma, oligodendroglioma and
vascular malformation.30–38
The origin of the MA lesion is not clear. Basically, it
was considered a hamartomatous proliferation of meningothelial cells, blood vessels and fibroblasts in variable
proportions.11,39 Foci of meningothelial hyperplasia are
known to appear in the context of von Recklinghausen’s
disease,40 and this could be a plausible origin for those
cases of MA associated with neurofibromatosis. On the
other hand, the observation of abnormal local vascularization in some cases has led several authors to regard MA
simply as a vascular malformation with added meningothelial reaction.33 However, recent immunohistochemical
studies did not support a meningothelial origin for the
perivascular cells, but suggest that pluripotent cells may
differentiate into various cell types found in MA.12,15,41–43
Immunohistochemistry has limited diagnostic value as
staining patterns vary among MA cases. Our immunostaining results parallel those in the literature. Only
vimentin, as a non-specific marker of the mesenchymal cell
was consistently positive. EMA expression, as a marker
for arachnoid cap cells was noted in two of the five cases.
These findings indicated that perivascular mesenchymal
components, such as fibroblasts and perivascular connective tissue might be playing an important role in the pathogenesis of MA.
Neurofibrillary tangles have been described in a wide
spectrum of diseases and are seen in the setting of degeneration within neuronal cytoplasm. These changes were
identified in only one of our five cases. In the largest
reported series of MA, Halper et al.13 described the presence of NFT within cortical neurons in five of their six
reported cases. Only rarely have tangles been noted by
others in the literature.12,41,44 Therefore, Halper and his
colleagues hypothesized that the presence of NFT in
MA more likely represented a secondary degenerative
phenomenon rather than an intrinsic component of the
lesion.13
Finally, the diagnosis of MA is important since it
is a hamartomatous condition that presents as a massoccupying lesion within the cortex. It should be considered
in differential diagnosis of intracortical lesions, especially
in children and young adults. The superficial location,
perivascular cuffs of spindle-cell proliferation with variable
involvement of the overlying leptomeninges and calcification, and small plump vessels, should clinch the diagnosis.
It is important to make a correct diagnosis in order to avoid
further aggressive treatment.
© 2006 Japanese Society of Neuropathology

Sporadic meningioangiomatosis

ACKNOWLEDGMENTS
The authors thank Prof Yoichi Nakazato, First Department
of Pathology, School of Medicine, Gunma University,
Gunma, Japan for the consultation of case 5.

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