Acta Neurochir (2015) 157:841–853
DOI 10.1007/s00701-015-2375-y

CLINICAL ARTICLE - BRAIN TUMORS

Sporadic meningioangiomatosis with and without meningioma:
analysis of clinical differences and risk factors for poor
seizure outcomes
Chao Zhang & Yao Wang & Xiu Wang & Jian-Guo Zhang &
Jing-Jun Li & Wen-Han Hu & Kai Zhang

Received: 27 November 2014 / Accepted: 19 February 2015 / Published online: 11 March 2015
# Springer-Verlag Wien 2015

Abstract
Background Meningioangiomatosis (MA) is a rare cerebral
lesion. Sporadic MA occasionally combines with meningioma
(MA-M). The aim of the present study was to clarify whether
MA-M and pure MA have clinical differences and to determine risk factors for unsatisfactory seizure outcomes in sporadic MA.
Methods We reported 14 sporadic MA cases in our center and
conducted a literature review. We compared the demographic,
clinical, imaging, electrophysiological and pathological features and surgical outcomes. Logistic regression analysis
was performed to evaluate the risk factors for poor seizure
outcomes.
Results MA-M cases showed a more prominent male predilection (4.2 times vs. 1.6 times, p=0.04), a shorter duration of
symptoms (2.8±0.8 years vs. 5.2±0.6 years, p=0.02), and a
lower seizure incidence (53.6 % vs. 89.3 %, p<0.001) as

compared to pure MA. A gyriform alteration on imaging
was exclusively associated with pure MA. The Ki-67 was
higher in the meningioma component than in the MA component in MA-M (1.2±0.3 % vs. 6.1±1.1 %, p<0.001). Lesions
located in the temporal lobe predicted poor seizure outcomes
(p=0.02, OR=4.4, 95 % confidence interval, 1.24–15.89).
Conclusion Clinical differences may be caused by the different biological natures. MA-M seems to be a neoplastic lesion,
while pure MA seems to be a non-neoplastic lesion. Longterm follow-up is required for MA-M. Because the coexistence of hippocampal sclerosis may explain the poor seizure
outcomes of MA located in the temporal lobe, it is important
to identify underlying hippocampal sclerosis and to perform
complete resection.
Keywords Meningioangiomatosis . Meningioma . Seizures .
Gyriform

Chao Zhang and Yao Wang contributed equally to this work
C. Zhang : X. Wang : J.<G. Zhang : K. Zhang (*)
Department of Neurosurgery, Beijing Tian Tan Hospital, Capital
Medical University, No. 6, Tian Tan Xi Li, Beijing 100050, China
e-mail: zhangkai62035@163.com
C. Zhang : J.<G. Zhang : W.<H. Hu : K. Zhang
Stereotactic and Functional Neurosurgery Laboratory, Beijing
Neurosurgical Institute, Capital Medical University, Beijing 100050,
China
C. Zhang : J.<G. Zhang : W.<H. Hu : K. Zhang
Beijing Key Laboratory of Neurostimulation, Beijing 100050, China
Y. Wang
Beijing Jingmei Group General Hospital, Beijing 102300, China
J.<J. Li
Department of Pathology, Beijing Neurosurgical Institute, Capital
Medical University, Beijing 100050, China

Introduction
MA is a rare cerebral lesion characterized by a plaque-like or
nodular mass within the leptomeninges and the underlying
cerebral cortex. The main histopathological features of MA
are leptomeningeal meningothelial proliferation and
meningovascular proliferation [1]. MA can occur sporadically
or in association with neurofibromatosis type two (NF2). Sporadic MA clinically presents intractable seizures or vague neurological complaints (headache, nausea). Occasionally, sporadic MA combines with a neoplasm, most commonly meningioma (MA-M) [2]. MA is usually considered a benign, nonneoplastic lesion, while genetic and molecular studies have
suggested that MA-M may be a neoplasm [3–5]. Given the

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neoplastic nature of MA-M, it is reasonable to assume that the
clinical features of MA-M may be different from those of pure
MA. However, studies undertaking comprehensive comparisons of MA-M and pure MA are almost entirely lacking. In
this article, we analyzed 14 sporadic MA cases collected in
our center and 160 cases reported previously [1, 3–79]. We
compared the demographic, clinical, electrophysiological, imaging and pathological features, as well as the surgical outcomes, between the MA-M and pure MA groups. Clinical
features that influenced seizure outcomes were also assessed.

Materials and methods
Patients
A total of 14 sporadic MA patients (7 MA-M cases and 7 pure
MA cases) were treated in the neurosurgery department of our
center between 2009 and 2013. All of the patients were initially operated on in our center. The information, including the
types and duration of the seizures, was obtained from the
patients’ charts and the database. Each patient had at least
one preoperative brain magnetic resonance imaging (MRI)
and long-term video electroencephalography (EEG). The preoperative evaluation was conducted by a senior neurologist
and senior neurosurgeon. All patients underwent surgery by
three senior neurosurgeons in our center. Seizure and MA
outcomes were followed up. All protocols were reviewed

Table 1

and approved by the institutional review board of Beijing Tian
Tan Hospital.
Literature analysis
We conducted a literature search of the Embase and PubMed
databases until 2013, using the following subject headings
(MeSH) and keywords: meningioangiomatosis and
meningioangiomatosis. After reviewing abstracts, MA-M
cases and pure MA cases were retrieved. Case selection was
based on the full text, and all duplicate cases were discarded.
A comparison concerning demographic features, clinical presentation, location of MA, imaging, EEG, immunohistochemistry and surgical outcomes was performed between MA-M
cases and pure MA cases. Whether the patient had meningioma or not, age, sex, symptom duration and MA location were
evaluated for their influences on seizure outcomes.
Statistical methods
Descriptive statistics were applied to analyze the distributions
of demographic and clinical data. The t test was performed for
continuous variables and the chi-square or Fisher’s exact test
for discrete variables. Analysis of seizure outcomes was evaluated by binary logistic regression; seizure outcomes were
divided into seizure-free (Engel I) and unsatisfactory (Engel
II/III/IV) groups. All of the data were analyzed using SPSS
software (SPSS, version 18.0 for Windows; SPSS Inc.,

Summary of clinical features and surgical outcomes of 14 sporadic MA cases in our center

Case no. Sex/age (years old)/ Symptoms
duration of symptoms
(months)

Location

Histologic type
of meningioma

Ki-67 proliferation Removal Follow-up time
index (%)
(months)/seizure
outcome/MA outcome
MA Meningioma

1
2
3
4
5
6
7
8
9
10
11
12
13
14

Right frontal lobe
Right temporal lobe
Corpus callosum
Left parietal lobe
Right parietal lobe
Third ventricle
Left temporal lobe
Right frontal lobe
Right temporal lobe
Right parietal lobe
Right temporal lobe
Right parietal lobe
Anterior cranial fossa
Right temporal lobe

Transitional
Transitional
Transitional
Transitional
Fibrous
Fibrous
Psammomatous
No meningioma
No meningioma
No meningioma
No meningioma
No meningioma
No meningioma
No meningioma

0.8
1.0
2.5
5.0
1.3
2.5
2.0
3.2
1.1
1.5
2.2
5.0
2.2
1.1

M/30/1.5
M/32/180
M/3/0.3
M/12/24
M/23/144
M/13/24
M/23/18
F/10/108
F/25/7
F/26/84
F/5/0.6
M/10/0.6
M/3.5/10
M/27/156

Headache
GTCS
CPS
CPS
GTCS
Diabetes insipidus
CPS
GTCS
ABS+GTCS
GTCS
GTCS
GTCS
GTCS
GTCS

3.1
5.3
8.2
8.2
4.3
5.5
5.0

Total
Total
Total
Total
Total
Partial
Total
Total
Total
Total
Total
Total
Total
Total

24/SF/NR
39/SF/NR
20/SF/NR
12/SF/NR
20/SF/NR
dead
10/SF/NR
58/SF/NR
33/SR/NR
46/SI/NR
22/SF/NR
20/SF/NR
12/SF/NR
10/SI/NR

ABS absence seizure, CPS complex partial seizure, GTCS generalized tonic-clonic seizure, F female, M male, NR no recurrence, SF seizure free, SI
seizure improved

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Chicago, IL, USA). p-values<0.05 were considered statistically significant.

843

patients. In the other five patients, epileptic discharges were
recorded within MA lesions and the adjacent cortex. Ten of
the 12 patients with seizures were seizure free postoperatively.
No tumor recurrence occurred.

Results
Illustrative cases
Patients
Case 3
Our records yielded 14 patients, including 7 patients with pure
MA and 7 patients with MA-M (Table 1). The imaging characteristics were quite variable and nonspecific. Gyriform alterations on computed tomography (CT) and MRI were found
in cases 8 and 9 (Fig. 1). During surgery, MA presented diverse textures, colors and blood supplies. Most of the lesions
were located in the cortex, but some were found in the white
matter (case 12), third ventricle (case 6) and corpus callosum
(case 3). The electrocorticography (ECoG) data showed that
spikes and sharp waves originated from MA lesions in seven

Fig. 1 a Case 8: Axial CT showing a hyperdense lesion located in the
right frontal lobe with calcification. b Case 8: Axial CT revealing a
hyperdense mass with gyriform alteration. c Case 8: Axial T2-weighted
image demonstrating a gyriform hypointense lesion in the right frontal
lobe. d Case 9: Axial T2-weighted image presenting a gyriform lesion in

MA-M patient with the lesion located in the corpus
callosum A 3-year-old boy suffered from complex partial seizures for a month. There was no history of perinatal problems,
febrile seizures or developmental delay. MRI revealed an intracranial mass measuring 2×1.5×1.5 cm located in the corpus callosum. The lesion was hypointense on T1-weighted
imaging (T1WI) and hyperintense on T2-weighted imaging
(T2WI), with obvious homogeneous enhancement (Fig. 2a
and b). A soft yellow mass was completely removed, and at

the right temporal lobe. e Case 9: Axial T1-weighted imaging showing
inconspicuous enhancement within the lesion. f Case 9: Coronal T2weighted image demonstrating a right temporal lesion with gyriform
alteration

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Fig. 2 Case 3. a, b Enhanced MRI showing homogeneous enhancement
located in the corpus callosum. c Postoperative 3-month MRI (T2WI)
indicating total removal of the lesion and no recurrence. d Transition
zone between the MA component and meningioma component

(asterisks). Hematoxylin and eosin, original magnification×100. e The
histological appearance of the MA component. Hematoxylin and eosin,
original magnification ×100. f Ki-67-positive cells (arrows) in the
meningioma component, original magnification×100

the 3-month follow-up the MRI showed no recurrence
(Fig. 2c). After histopathological examination, MA-M was
confirmed, and a transition zone between the MA component
and meningioma component was observed (Fig. 2d and e). Ki67 was observed in 2.5 and 8.2 % of the cells of the MA and
meningioma, respectively (Fig. 2f). Preoperatively, ictal EEG
showed bilateral frontal and temporal epileptic activities
(Fig. 3). The patient was seizure free at last follow-up (Fig. 4).

the 3 month follow-up the lesion presented no recurrence
(Fig. 5 d). Under microscopy, the specimen showed numerous
small vessels ensheathed by spindle-shaped cells (Fig. 5e).
The cortical parenchyma showed reactive astrocytes associated with gliosis (Fig. 5f). Ki-67 was detected in less than 5 % of
the cells. Preoperatively, EEG indicated a right occipital and
temporal slow wave (Fig. 6). The patient was free of seizures
at last follow-up (Fig. 7).

Case 12

Histopathology

Pure MA patient with MA located in the white matter A 10year-old boy presented with a 20-day history of simple partial
seizures. There was no history of febrile seizures. CT revealed
a hypodense round mass with calcification in the right parietal
lobe (Fig. 5a). MRI revealed that the lesion was located in the
white matter without extension into the cortex. Ring-like enhancement was observed (Fig. 5b). The lesion was
hypointense with a central cerebrospinal-like area on TIWI
and hyperintense on T2WI (Fig. 5c). During surgery, a soft,
lightly vascularized lesion was completely removed, and at

Specimens demonstrated numerous spindle-shaped cells arranged in fascicles or in a storiform pattern surrounding the
small blood vessels, with or without psammoma bodies. The
nuclei of the spindle-shaped cells were normal, and no features or mitosis was found. The histological type of the meningioma component was variable (Table 1). Vim, CD34 and
GFAP were positive in most cases, while other markers were
expressed differently. Ki-67 was higher in meningioma components than in MA components in MA-M cases (2.2±0.5 %
vs. 5.6±0.7 %, p<0.001).

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Fig. 3 Ictal EEG in case 3 shows bilateral frontal and temporal epileptic activities

Literature review

Location of MA

A total of 160 sporadic MA patients (35 MA-M cases and 125
pure MA cases) were collected in 75 previous publications.
Combined with our 7 MA-M cases and 7 pure MA cases, 174
patients were analyzed as follows (Fig. 8).

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. In addition to the posterior head, the cingulate gyrus,
corpus callosum, third ventricle and brain stem were occasionally involved. No significant difference was found in the location between the MA-M and pure MA (Table 2).

Demographic features
The mean ages at the time of diagnosis were nearly identical
for MA-M and pure MA. A more obvious male predilection
was found in the MA-M group. In addition, the MA-M cases
showed a shorter duration of symptoms before diagnosis
(Table 2).
Clinical presentation
As the most common symptom, seizures were found in 116
patients (79.5 %). The MA-M group presented a significantly
lower incidence of seizures than the pure MA group (Table 2).
There were no differences in seizure types between MA-M
and pure MA cases (Table 2). Other atypical symptoms included headache, vomiting, diabetes insipidus, facial weakness, muscle atrophy and pain.

Imaging
The most common finding on CT was a round, hypodense
mass with varying degrees of calcification, occurring in 49
of 95 (51.6 %) patients (Table 2). On MRI, the lesions were
confined to the cortex in 90 (88.2 %) of 102 patients. On
T1WI, MA usually demonstrated a low- or isointense signal,
occasionally with a mixed signal. On T2WI, 73.2 % lesions
were hyperintense, while the others were hypointense. Gadolinium enhancement was applied in 65 patients, of whom 50
(76.9 %) showed minimal enhancement to substantial enhancement. There were no significant differences in imaging
features between MA-M and pure MA (Table 2). However, A

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Fig. 4 Postoperative EEG in case 3 is normal with no epileptic activity

total of 17 patients [1, 10, 18, 32, 45, 69, 78, 80], including
case 8 and case 9 in our series (Fig. 1), showed a gyriform
signal pattern on either CT or MRI, which were all pure MA
patients.
EEG
Fifty-four patients underwent scalp EEG, and 41 of them
(75.9 %) showed congruence on the location of epileptiform
discharges and MA lesions. The remainder of the patients
presented multifocal spikes, generalized spike waves or sporadic spikes. ECoG data, obtained from 18 patients, recorded
spike or sharp waves in accordance with MA in 9 patients
(50 %), whereas, the other 9 (50 %) patients showed spikes
originating from the surrounding cortex of the lesion. There
were no differences in ECoG data between the MA-M and
pure MA cases (Table 2).
Histopathology and immunohistochemistry
Histopathologically, the main features of MA were
leptomeningeal meningothelial proliferation and
meningovascular proliferation. MA-M included features of

MA and meningioma; a transition zone between the MA and
meningioma components was sometimes presented. The meningiomas were mostly of the transitional subtype (Table 3).
Immunohistochemically, Vim was expressed in nearly all
of the MA cases: 38 (92.7 %) of 41 MA were Vim positive.
Other immunostaining results, including for CD34, EMA,
GFAP and S-100, were heterogeneous. There were no differences in Vim, CD34, EMA, GFAP and S-100 between the
MA-M and pure MA groups (Table 4). Ki-67 was consistently
low in MA (ranging from 0.1 to 15 %). There were no differences in Ki-67 between the MA-M and pure MA groups (1.2±
0.3 % vs. 1.4±0.5 %, p=0.67) (Table 4). However, in MA-M,
Ki-67 was higher in the meningioma component than in the
MA component (1.2±0.3 % vs. 6.1±1.1 %, p<0.001).
Outcomes after surgery
Only 2 patients had MA recurrence after surgery of a total of
111 patients [33, 39]. Seizure outcomes were followed up in
102 patients, with 89 (87.2 %) patients becoming seizure free
(Engel I). Data obtained from 51 patients regarding the extent
of surgery and the seizure outcome revealed that the seizurefree rate (88.6 %, 39 of 44) with total resection was slightly

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Fig. 5 Case 12. a CT scan revealing a hypodense round mass with
calcification in the right parietal lobe. b Enhanced MRI scan showing
enhancement of the lesion with surrounding hypointensity. c MRI
showing hyperintensity of the lesion on T2WI. d Postoperative 3-month
MRI (T2WI) indicating total removal and no recurrence of the MA lesion.

e Proliferating blood vessels are ensheathed by spindle cells.
Hematoxylin and eosin, original magnification×100. f At the boundary
of the MA in the white matter, gliosis is observed (asterisks).
Hematoxylin and eosin, original magnification×40

higher than that (71.4 %, 5 of 7) with partial resection, but
with no significant difference (p=0.24). Congruence on location of epileptiform discharges and MA on ECoG, as well as
seizure type, had no correlation with seizure outcome (p=
0.63; p=0.92). Additionally, the age, sex, MA size and duration of symptoms and whether MA was combined with a
meningioma were not correlated with seizure outcomes. The
location of the MA had a marked effect, with lesions in the
temporal lobe indicating an unsatisfactory seizure outcome
more often than extratemporal lesions (p = 0.02, OR = 4,
95 % confidence interval, 1.24-15.89) (Table 5).

occurred sporadically [72]. Occasionally, MA has been reported to coexist with meningiomas [11, 39, 54, 82], arteriovenous
malformations [23], encephaloceles [75], oligodendrogliomas
[50], meningeal hemangiopericytomas [39] and focal cortical
dysplasia [65]. Among these, MA with meningioma is the
most frequent combination. MA occurs mainly in children,
adolescents and younger adults suffering from intractable seizures and headache [4, 39]. A previous study suggested that
MA-M affected a younger age group than pure MA [15].
However, a similar predilection for young adults, as well as
broad age distributions of MA-M patients and pure MA patients, was found. 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. Rarely, MA has been documented to occur in the

Discussion
MA is a rare, focal intracranial lesion that was first described
by Bassoe and Nuzum in 1915 as an incidental autopsy finding in a patient with neurofibromatosis-2 (NF2) [81].
Takeshima et al. demonstrated that only 25 % of MA cases
were associated with NF2, while in the remaining 75 %, it

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Fig. 6 EEG in case 12 shows slow waves in the right occipital and temporal regions

diencephalon [25], in the brain stem [43] and outside the cerebral cortex [54]. In our series, we reported an MA-M lesion
(case 3) that was believed to be the first reported MA-M case
involving the corpus callosum (Fig. 3).
The variety of imaging findings of MA usually leads to
erroneous diagnoses [1, 80]. 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]. MA is believed to
be mainly confined to the cerebral cortex; the subcortical
white matter is rarely involved [1, 45, 74]. Recently, an excellent correlation between sporadic MA located in the subcortical whiter matter and hyperintensity on T2WI was established
by Jeon et al. [35]. With our imaging findings, this supports
the notion that MA may involve the subcortical white matter
as well as cortex [35].
It is difficult to distinguish between pure MA and MA-M
on imaging. 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). This finding could be explained by the
thickened cortex, where abundant proliferating
leptomeningeal microvessels and spindle-shaped cells were
located. However, if the lesion is completely calcified, it might
present hypointensity without gyriform alteration on MRI
[80]. Although the reason why gyriform changes were only
found in pure MA is unclear, such gyriform alteration could
help to distinguish pure MA from MA-M.
Immunohistochemistry has no specific diagnostic value for
MA [41]. Only Vim, as a nonspecific marker of mesenchymal
cells, was consistently positive [1, 74]. Interestingly, we found
that the majority of MAs were CD34 positive (23 of 33). This
finding suggests that, as an endothelial cell marker to detect
microvascular proliferation activity, CD34 could be used as an
ancillary method for identifying sporadic MA. As a proliferation index, Ki-67 was consistently low in MA; however, all of
the literature pertaining to Ki-67, as well as our cases, has
found that Ki-67 is higher in the meningioma component than
in the MA portion [3–5, 61]. One reasonable explanation is
that meningioma arises from the MA component with an

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Fig. 7 Postoperative EEG in case 12 is normal with no epileptic activity

additional genetic alteration, which may confer more proliferation potential on it.
Overall, the pathogenesis of MA is unclear. A presumed
hamartomatous or non-neoplastic nature is favored by its typically benign clinical course and the absence of marked proliferation activity in most cases [15]. For pure MA, genetic

Fig. 8 Participant flow diagram

studies have supported that the majority of pure MA lesions
are non-neoplastic, based on no evidence of NF2 gene deletions [4, 5, 84]. However, a few cases have shown loss of
heterozygosity on 22q12 and 9p21, raising the possibility of
a neoplastic nature [3, 72]. For MA-M, meningiomaassociated genetic alterations have always been found in both
the MA and meningioma components, substantiating the neoplastic nature of MA-M [3–5]. Based on the aforementioned
genetic studies, we postulate that pure MA and MA-M have
distinct biological natures, with pure MA lesions more similar
to non-neoplastic lesions and MA-M more similar to neoplastic lesions, which can result in different clinical features. Our
findings supported this notion. Compared to pure MA, MA-M
patients showed a lower seizure incidence. It is believed that
the type of tumor plays an important role in epileptogenesis.
Developmental lesions have a greater likelihood of causing
seizures than neoplastic lesions because these lesions consist
of well-differentiated cells that can release neurotransmitters
or modulators with a role in epileptogenesis [85]. Therefore,
the difference in pathogenesis might explain the discrepancy
in seizure frequency between MA-M and pure MA. Moreover,
MA-M cases showed a shorter symptom duration before

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Table 2

Acta Neurochir (2015) 157:841–853
Comparison of MA-M and pure MA

Parameter

Patient age (years)
Gender (M:F ratio)
Symptom duration (years)
Seizures incidense
Seizure types
Partial seizures
Generalized and other types of seizures
Location
Frontal lobe
Temporal lobes
Other locations
Imaging
Hypodense mass with calcification
on CT
Hyperintense on T2WI
Enhancement on MRI

Table 4 Comparison of the immunohistochemical markers of MA-M
and pure MA

MA-M
(n=42)

Pure MA P value
(n=132)

Markers (positive ratio)

MA-M

Pure MA

P value

17.4±2.5
4.2:1
2.8±0.8
53.6 %

20.0±1.3
1.6:1
5.2±0.6
89.3 %

p=0.37
p=0.04
p=0.02
p<0.001

Vim
CD34
EMA
GFAP

88.9 %
78.6 %
66.7 %
50.0 %

95.7 %
63.2 %
51.7 %
50.0 %

p=0.83
p=0.57
p=0.31
p=1.00

p=0.38

S-100
Ki-67

54.5 %
1.2±0.3 %

47.1 %
1.4±0.5 %

p=1.00
p=0.67

61.5 %
38.5 %

52.4 %
47.6 %

42.0 %
38.7 %
19.3 %

29.7 %
33.9 %
36.4 %

53.3 %

51.3 %

p=0.55

60.0 %
84.6 %

71.7 %
75.0 %

p=0.30
p=0.37

Gyriform alteration
0
Congruence on location of epileptiform 60.0 %
discharges and lesions on EcoG
Seizure free
92.3 %

17
46.2 %

p=0.50

85.7 %

p=0.45

p=0.18

diagnosis. One potential explanation for this difference is that
with a neoplastic biological behavior, MA-M caused progressively intolerable symptoms and eventually led to surgery at
early stages. Given the neoplastic nature of MA-M, a more
systematic and longer term follow-up might be favored in
future clinical practice.
Surgery prompts favorable seizure outcomes for MA; however, seizures persisted in a proportion of patients, and more
than 70 % patients required continuous antiepileptic drug treatment [1]. Although ECoG findings demonstrated a range of
electrophysiological abnormalities, complex epileptogenicity
did not show a correlation with poor seizure outcomes. We
hypothesize that this finding was due to small sample bias, with
only 18 cases available for ECoG data. We believe that it is still

necessary to perform ECoG covering MA and the adjacent
cortex during surgery. Except for lesions located in the temporal
lobe, no other factors emerged as a determinant of seizure outcomes, including age, sex, MA size, duration of symptoms and
the presence or absence of meningioma (Table 5). A potential
explanation may be that early onset seizures due to temporal
sporadic MA kindle the hippocampus and finally result in dual
pathology [86]. Dual pathology refers to the coexistence of
hippocampal sclerosis and an extrahippocampal lesion, in
which the lesion most commonly involves the temporal lobe
[87]. Moreover, the hippocampus is more vulnerable in early
childhood, and the dual pathology was almost exclusively
found in patients whose lesions were congenital or occurred
early in life and have epileptogenic potential [88]. Sporadic
MA perfectly matches such features of extrahippocampal lesions with dual pathology, and temporal MA with hippocampal
sclerosis has been reported [1]. However, because subtle microscopic malformations of the hippocampus may not show abnormalities on MRI, underlying hippocampal sclerosis may has
been underestimated. Hence, temporal lobe MA should draw
closer attention in future clinical practice. Volumetric MRI, stereotactic EEG and ECoG during surgery could be valuable in
identifying subtle hippocampal sclerosis in temporal sporadic
MA.
In conclusion, compared to pure MA, MA-M presents with
a more prominent male predilection, lower seizure incidence
and shorter symptom duration. Such clinical differences, combined with previous genetic findings, substantiate the possibility that MA-M is more similar to a neoplasm and that pure

Table 3 Summary of histopathological features of the meningioma of
the MA-M
Histopathological subtypes of
the meningioma in MA-M

Number in total (literature
and our cases, n=42)

Transitional
Fibroblastic
Meningothelial
Microcystic
Atypical
Psammomatous
Metaplastic

23
9
4
2
2
1
1

Table 5 Odds ratios of potential risk factors for poor seizure outcomes
(Engel II/III/IV) in sporadic MA patients undergoing surgery (n=87)
Risk factors

p-value

OR

95 % CI

Sex
Age
Duration of symptoms
MA-M
Temporal location

0.137
0.435
0.754
0.998
0.022

0.387
1.023
1.001
0.000
4.435

0.111–1.354
0.967–1.082
0.993–1.010
0.000–
1.238–15.886

CI confidence interval, OR odds ratio

Acta Neurochir (2015) 157:841–853

MA is more similar to a non-neoplastic lesion. Gyriform alterations on imaging can help to distinguish pure MA from
MA-M. In addition, MA-M should be differentiated from malignant childhood meningioma, and a long-term follow-up is
required due to its potential neoplastic nature. Given the unsatisfactory seizure outcomes of MA located in the temporal
lobe, which can be associated with dual pathology, recognition of underlying hippocampus sclerosis and the performing
of complete resection are critical for temporal sporadic MA.
Acknowledgments This project was funded by grants from a highlevel specialist training project of the Beijing Health Bureau (no. 20113-029) and Beijing Municipal Administration of Hospitals Clinical Medicine Development of Special Funding (no. ZYLX201305).
Conflicts of interest All authors certify that they have no affiliations
with or involvement in any organization or entity with any financial
interest (such as honoraria; educational grants; participation in speakers’
bureaus; membership, employment, consultancies, stock ownership, or
other equity interest; and expert testimony or patent-licensing arrangements) or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials
discussed in this manuscript.

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Comments
Zhang and colleagues analyzed the clinical and morphological data and
treatment results of 14 patients affected by a rare cerebral disease characterized by a leptomeningeal meningothelial proliferation called
Bmeningioangiomatosis.^ Such meningeal abnormality was identified
by the authors in seven cases as an isolated disease and in seven further
cases associated with a meningioma. The authors also analyzed 160 similar cases reported in the literature to date. According to the results presented in the study, meningioangiomatosis should be considered a nonneoplastic lesion that may, however, be associated with epilepsy. Nonetheless, the contextual presence of other epileptogenic foci (i.e.,
mesiotemporal sclerosis) is common, and some of the lesions may be
associated with gyriform alteration or enhancement. Accurate epilepsy
preoperative assessment is therefore mandatory to gain treatment success.
Meningiomas associated with menigioangiomatosis are typical neoplastic
lesions, requiring standard follow-up. The study provides interesting information on the histopathological features of these lesions and the differences from meningiomas. On such bases, it can be suggested that when
meningioangiomatosis is associated with meningiomas, these tumors
could arise from the non-neoplastic meningioangiomatosis lesions by
additional genetic alterations, which may confer a greater proliferation
potential.

Alfredo Conti
Messina, Italy