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Revista Chilena de Neurocirugía 37 : 2011
Intraventricular Meningiomas in Adults - Clinical
Series and Review of the Literature
Paulo Henrique Pires de Aguiar 1, Adriana Tahara4, Celso Agner 2, Marcos Vinicius Calfat Maldaun1,
Alexandros Theodoros Panagopoulos3, Toshinori Matsushige4, Kaoru Kurisu4
1. Department of Neurology, Division of Neurosurgery - Hospital das Clínicas - University of São Paulo Medical School - São Paulo, Brazil;
2. Division of Neurosurgery - Albany Medical Center, Albany, New York; 3. Division of Neurosurgery - Santa Casa Medical School - São Paulo,
Brazil; 4. Hiroshima University Hospital - Hiroshima, Japan.
Rev. Chil. Neurocirugía 37: 23-28, 2011
Abstract
Background: Intraventricular meningiomas are rare tumors and pose clinical, radiological, and surgical challenges. Individualized approach helps to establish successful results. Methods: Thirteen patients underwent craniotomy for intraventricular
meningioma resection from 1999 to 2007. The mean age was 45 years (23-64), time of presentation between 25 days to three
years. There were ten females and three males. Headaches and seizures were the most frequent initial presentations. Tumors
were located in the ventricular trigone in 11 patients and in the temporal horn in two. Results: There were seven posterior temporal and seven parieto-occipital transcortical craniotomies, one patient was operated two times. Resection grade was Simpson
I in nine patients, Simpson II in four, and Simpson III in one case. Surgical mortality was zero. There were six complications.
Two patients had ventriculitis, one patient had hematoma of the surgical bed, one patient had severe post-operative cognitive
impairment and one presented with progression of motor deficits. In two patients, there was transient memory disturbance after
the parieto-occipital approach. Conclusion: Correct understanding of microsurgical anatomy cooperates for further success in
operation of intraventricular meningiomas. Pre-operative embolization is helpful to reduce bleeding when a suitable tumor feeder
can be accessed with no reflux. Dynamic changes in the shape of the ventricular cavity have to be considered when planning the
most suitable route. Rigorous hemostasis and ventricular drainage are important points to avoid main complication.
Key words: meningiomas, intraventricular, embolization, surgery.
Introduction
Intraventricular tumoral location is rare
and accounts for 0.5% to 5% of all intracranial meningiomas (1-16). As compared
to other intraventricular tumors, meningiomas are responsible for 20 to 30% of
the cases (17) and its incidence is higher
in childhood and adolescence. Although
meningiomas constitute only 1% to 4%
of all intracranial tumors in pediatric age,
intraventricular location is observed in
9.4% to 22% of all children (5,18-20). There is marked female predominance in all
series presented (3,7,10,13,14,16,19,21-23).
Since intraventricular meningiomas are
slow-growing tumors, they are generally
large and silent at presentation (9,14,15,24-28).
The most common location is the lateral
ventricles, although they can be appreciated in the third and fourth ventricles
as well (1,6,29-40). Reported topographic
distribution is 77.8% in the left lateral ventricle atrium, 15.6% in the third ventricle,
and 6.6% in the fourth ventricle (41).
We present our clinical series and discuss
current literature in support of avoidance
of complications, technical surgical and
radiological approaches to these tumors.
Material and methods
Clinical materials
We analyzed primary intraventricular
meningiomas in adults. In our series, we
excluded falcine and tentorial secondary tumors.
At Hospital das Clinicas of the University
of Sao Paulo, Division of Neurosurgery,
we operated thirteen patients with diagnosis of intraventricular meningioma
from 1999 to 2007. The Pediatric Neurosurgery group in our Institution manages all the meningiomas at their res-
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Revista Chilena de Neurocirugía 37 : 2011
pective age group. We excluded those
patients from our series.
The mean age was 45 years old (2364 years old). There were ten females
and three males. Duration of symptoms
was between 25 days and three years.
All tumors were located in the lateral
ventricles. Headache was present in
12 patients. In four, there were signs
of intracranial hypertension (vomiting).
Three patients presented with cognitive impairment at onset. Three patients
presented with motor impairment, manifested as hemi or monoparesis. Four
patients presented with seizures. Papilledema was seen in seven patients,
corticospinal signs in six, and homonymous hemianopia in four. Tumors were
predominant on the left hemisphere (9
patients), in the ventricular trigone in 11
patients, in the temporal horn in two. In
many occasions, diagnosis was delayed since complaints were transient or
episodic. Complete clinical, radiological,
histological and surgical characteristics
are shown in Table 1.
On brain MRI, isointense signal was
seen on T1 in ten patients, hypo signal
in three, and Gadolinium enhancement
on T1 in all patients. MRI angiography
showed increased vascularization in
10 patients and hypo vascularization in
three. In all patients, including the ones
with tumors within the temporal horn,
there was enlargement of the posterior
choroid artery.
Results
Fourteen surgeries were performed
in thirteen patients. There were seven
posterior temporal and seven parietooccipital transcortical craniotomies. Resection grade was Simpson I in nine cases, Simpson II in four, and Simpson III
in one case. The patient with Simpson III
resection grade underwent reoperation.
There was no mortality. Complications
were seen in 8 of 14 cases. Two patients
had ventriculitis, one hematoma in the
surgical bed, one had severe cognitive
24
impairment post-surgery, and one presented with additional motor deficits.
In two patients, there was transient
memory disturbance after the parietooccipital approach.
Discussion
Symptoms and Signs
The histological origin of intraventricular
meningiomas is uncertain. The choroid
plexus stroma or remains of arachnoid
within the choroid are the most likely
histological origins of these tumors and
may explain their predominant topography within the ventricular trigon (10,13,37).
Most of the clinical symptoms of intraventricular meningiomas are related to
increased intracranial pressure. Mass
effect due to direct pressure on adjacent
brain structures is another common
clinical manifestation. They are slow
growing tumors and reach a substantial size prior to becoming symptomatic.
Symptoms may occur earlier if the tumor
is located near a zone of cerebrospinal
fluid (CSF) outflow (10,13,37). On literature
review, duration of symptoms ranged
from a few days to several years. Cardinal symptoms were signs of increased
intracranial pressure (86%), followed by
corticospinal tract signs (43%), visual
field defects (36%), cognitive changes
(29%), and seizures (7%) (3,38,45). We had
similar clinical presentation in our case
series. There are few cases reported
of intraventricular meningioma in adults
(1,18)
. There is rare association with intracranial hemorrhage (42-44). Due to the
rarity of these tumors, autopsy findings
are scarce(45). Case seven in our series
presented with a primary intraventricular
hemorrhage. Post-surgical pathological
finding suggested a meningiothelial meningioma. The tumor was highly vascular with an increased pattern of angiomatous and cavernous vessels. Subarachnoid hemorrhage at presentation was
described in a few case series (44).
Differential diagnosis
The most important differential diagnosis is the Intraventricular solitary fibrous
tumor (46-49). Intraventricular meningioma
is the most common trigon intraventricular tumor in adults (49).
The differential diagnosis of lateral trigon
ventricular tumors and should include
choroid plexus papilloma in patients under 10 years of age; low-grade gliomas,
such as ependymoma, oligodendroglioma, and low-grade astrocytoma, in patients between 10 and 40 years of age;
and metastases and lymphoma after
the fourth decade of life (42,49,50).
Pathology
Histopathological features of these tumors are similar to those seen in meningiomas in other locations (4).
Bertalanfy found that most of intraventricular meningiomas were meningothelial, transitional (mixed), or lymphoplasmacyte-rich meningiomas (81%). Three
tumors were classified as atypical (19%)
and the MIB-1 proliferation index ranged
from 1% to 40% (3). In five of our cases,
MIB-1 ranged from 1% to 5%. In the
atypical tumors, the values were 13%
in average. The same percentages have
been demonstrated for meningiomas in
other locations (51, 52). MIB-1 was not evaluated in eight cases.
Rare pathological types, such as rhabdoid, osteoblastic and chordoid types
have been described (2,24,53). In our series
the majority were benign (12/13). There
were nine meningothelial meningiomas,
two transitional, and one fibrous. There was one atypical meningioma with
secondary malignant transformation by
occasion of the second surgery.
Pre-operative embolization
Pre-operative embolization of intracranial meningiomas has been performed
since the late 1960s (54). Better understanding of the angiographic anatomy,
abnormal connections between the
internal and external carotid artery systems, as well as potential communications between the carotid and vertebral
arteries allowed for a better understanding of the potential dangers associated
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Revista Chilena de Neurocirugía 37 : 2011
Table I:
Clinical, radiological, and surgical results present on patients with intraventricular meningiomas (F: Female; M: Male)
Case
Age/sex
Figure Number
Presenting symptoms Histology
Localization in ventricle
Surgery and grade of resection
1
28/F
Figure 2
Headache, mild hemiparesis,
Fibrous/Benign
Left trigone
Transcortical parieto-occipital and visual disturbance
approach,
2
59/F
Figure 3
Sudden onset of headaches,
Meningothelial/Benign Left trigone
Transcortical parieto-occipital
associated with primary
intraventricular
hemorrhage on head CT
3
29/M
Headache and vomiting
Meningothelial with
Right trigone
Posterior temporal gyrus approach,
extensive areas of
psamomas, Benign
Surgical Approach
Postoperative course
Simpson I
Simpson I
Ventriculitis+ transient
Gerztman syndrome
Simpson II
Transient memory deficit
Simpson I
No deficits
4
23/F
Headache+ seizure +
Meningothelial/ Benign Right trigone
Posterior temporal gyrus approach
Simpson I
vomiting
No deficits
5
54/F
Headache + seizure
Meningothelial/ Benign
Left trigone
Transcortical parieto-occipital approach
Simpson II
Transient memory deficit
6
48/F
Headache +vomiting
Meningothelial /Benign
Right trigone
Posterior temporal gyrus approach
Simpson II
No deficits
7
51/F
Figure 5
Headache + cognitive
changes + Hemiparesis +
visual disturbance
Meningothelial/ Benign
Left temporal horn
Posterior temporal gyrus approach
Simpson I
No deficits
8
56/F
Seizure + cognitive changes Transitional/ Benign
Left Temporal horn
Posterior temporal gyrus approach
Simpson II
+ visual disturbance
Figura 4
9
34/M
Figure 6
Headache + vomiting +
mild hemiparesis +
visual disturbance
Atypical with areas of
Right trigone and
Meningothelial at
Right temporal horn
first surgery,
First surgery, posterior temporal gyrus.
Second, transcortical
parieto-occipital
Simpson III first
surgery, Simpson I
second
Malignant at second
surgery (After 3 weeks)
Hematoma in surgical bed + Seizure + ventriculitis
Severe left hemiparesis
after the first surgery
(Hemiplegia)
No deficits
10
48/F
Headache and mild
Meningothelial/ benign Left trigone
hemiparesis
Transcortical parieto occipital
Simpson I
approach, Simpson I
No deficits
11
38/F
Headache+ seizure +
Meningothelial/ Benign Left trigone
mild hemiparesis
Transcortical parieto-
Simpson I
occipital approach
No deficits
12
48/F
Figure 7
Headache +
Meningothelial/Benign Left trigone
mild hemiparesis
Posterior temporal
Simpson I
gyrus approach
No deficits
Transcortical parieto-
No deficits
13
64/F
Figure 8
Headache +
Transitional/Benign
Left trigone
cognitive changes
with those therapies. When performed
for the right conditions, it allows better
tumoral resection with significant decrease in intra-operative hemorrhage.
Embolization of tumors supplied by intracranial vessels, in particular distal
carotid artery branches or the choroid
arteries carries the risk of embolization
of unwanted vascular territories and
development of permanent neurological deficits. Oyama et al, in 1992, described a successful embolization of a
fibroblastic meningioma fed primarily by
Simpson I
occipital approach
the anterior choroid artery. There was
a small amount of hemorrhage during
surgery and no permanent deficits after the combined therapy (55). Correct
understanding of the angiographic anatomy, possible unwanted anatomical
connections, and utilization of a precise
angiographic technique with the utilization of flow-directed catheters (smallest
available is 1.2F in external diameter),
the right embolic agent, and correct
surgical planning after embolization. Attention for agent reflux is a crucial aspect
of embolization, since the embolic agent
may suddenly occlude an important cortical vessel. Pre-operative embolization for
these tumors is, therefore, usually feasible.
Surgery
Bhatoe et al, 2006 presented a series of
12 intraventricular meningiomas (IVM).
In their experience, a parieto-occipital
(trigon) craniotomy should be performed
through for lateral ventricular, transcortical-transventricular route for third ventricular, and sub-occipital craniotomy for
fourth ventricular tumors (4). Trigon IVMs
are most commonly resected via intra-
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Revista Chilena de Neurocirugía 37 : 2011
parietal/inter-parietal or parietal-occipital approach (3). Neuronavigation based
in pre operative exams is very useful for
precise localization and avoidance of
neurological morbidity, but during the
tumor removal and dynamic brain shift
it is not suitable anymore. Since the majority of the tumors are on the dominant
side, determination of the best surgical
avenue is very helpful in achieving the
maximal surgical respectability with the
minimal risk. Bertalanfy et al utilized neuronavigation in 8/16 of their patients (3).
This was the preferred route for lateral
ventricular tumors, due to the shorter
distance from the cerebral cortex to the
tumor. If we cannot determine the shorter surgical route, we choose the temporal access for right-sided and parieto-occipital for left side lesions, avoiding
the dominant arcuate fascicle, involved
in cognitive tasks (Guideline 1).
We believe that the parieto-occipital
route for left lateral ventricular meningiomas mainly is a safe surgical approach
and is not commonly associated with
post-operative visual deficits. Neuronavigation and intra-operative ultrasound
have been excellent technological advances in the safe resection of these
lesions. Neuronavigation was utilized in
our last case and intra-operative ultrasound in eight of the 13 cases.
Intraventricular Meningiomas
Yes
No
Hidroccephalus
Perioperative Shurt
find the cortex where exists the shortes
disntence between the tumor
end cortical surface
No
Yes
Left
Approach is done by the tumor
Parieto occipital aproach
Right
Temporal approach
Figure 1: Surgical and clinical management algorithm of intraventricular meningiomas
Figure 2: Case 5 – 54 year old female patient with small left trigone meningioma. Figure
2A: Supine position and surgical view demonstrate occipito-parietal sulcus resection; Figure 2B:
After plan dissection, the tumor capsule is identified and the tumor is resected along its cleavage
plan, as demonstrated on the figure.
Piecemeal tumor removal can be easily
achieved. Special intra-operative attention should be paid to the choroid vessels (14). Once significant tumor debulking occurs, devascularization ensues
(15). There should be constant monitoring for development of hydrocephalus
or ventricular sequestration. Continuous
post-operative external ventricular drainage avoids some of the complications
appreciated with hydrocephalus (15).
Functional MRI is helpful in the surgical
planning and avoidance of the optical
tract (15,50,56,57). In our series, MRI
tractography was useful in planning the
surgical assessment between the optic
radiation and the splenium of the corpus
callosum in case 13.
26
Figure 3: Case 7 - 51 year old female patient. Figure 3A: Preoperative CT scan shows a large left
peri-cortical ventricular meningioma. The posterior temporal gyrus approach was chosen to avoid
cortical damage; Figure 3B: Postoperative CT shows complete tumor removal and small area of
pneumocephalus.
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Revista Chilena de Neurocirugía 37 : 2011
Route related complications
Transient memory disturbance after removal of an intraventricular trigon meningioma by a parietal-occipital inter-hemispheric
pre-cuneous approach was previously
described (58,59). We presented two cases
with transient memory disturbance after
parietal-occipital approach. Another patient presented with complete Gerstman
syndrome that lasted one year and partially recovered.
Outcome
One of our cases had the diagnosis of
atypical meningioma at first operation
and malignant recurrence on reoperation, three months afterward. Drop
subarachnoid metastasis of intraventricular meningiomas has been described
in the literature (8,60,61). The clinician
should continuous follow-up for development of further neurological symptoms
even though these tumors have a high
likelihood of benign outcomes.
Conclusions
Intraventricular meningiomas in adults
are rare. Suitable surgical plan based
on neuroradiological information and
correct judgment is the main point for
successful outcome. Main complication
can be avoided using ventricular drainage, rigorous hemostasis and careful
management of blood pressure.
Table II:
Review of the main case series in the literature. (F: Female, M: Male, N: Number of Cases, NA: Not Available)
Authors
Year Number
of Cases
Period
of Treatment
Mean Age
Gender
Left
(years)
Trigone on
left ventricle
Temporal
3rd
horn
Ventricle
Foramen Posterior fossa
of Monro or 4th Ventricle
Total
ressection
Nakamura
2003
16
1978-2001
41,7
8H/8M
13
11
0
1
2
0
15
Erman
2004
8
1995-2003
44,6
6F/2H
7
7
0
1
0
0
8
Main access
to trigone
Transcortical parietooccipital -11 cases
Transcortical parieto occipital -5cases
Posterior temporal gyrus -02 cases
Bertalanfy
2006
16
1980-2004
44
11F/5M
15
14
1
0
0
1
15
Transcortical parieto
occipital/intraparietal/interparietal
Transcortical parieto occipital
Bhatoe
2006
12
34,6
9F/3M
9
9
0
1
0
2
NA
2006
25
1989-2003
39
17F/8M
24
20
2
0
2
1
24
Liu
Lyngdoh
2007
9
1989-2003
34,6
5F/4M
7
7
0
2
0
0
8
Interparietal – 02 cases
Posterior middle temporal gyrus -05 cases
13
1999-2007
40,2
10F/3M
13
11
2
0
0
0
12
Present study 2008
Transcortical parieto-occipital-07
Posterior temporal gyrus -06
Transcortical parieto-occipital -0 cases
Recibido: 01.09.11
Aceptado: 01.10.11
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Corresponding Author:
Adriana Tahara
Hiroshima Ken Hiroshima Shi
Minami ku Kasumi 1-2-3
734-8551
Phone: 81 82 09086021909 (Japan)
Email: [email protected]
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