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Neurosurg Rev (2006) 29: 3035

DOI 10.1007/s10143-005-0414-5

ORIGINA L ARTI CLE

Alexander Bertalanffy . Karl Roessler .


Oskar Koperek . Ellen Gelpi . Daniela Prayer .
Markus Neuner . Engelbert Knosp

Intraventricular meningiomas: a report of 16 cases

Received: 9 March 2005 / Revised: 22 June 2005 / Accepted: 2 August 2005 / Published online: 24 September 2005
# Springer-Verlag 2005

Abstract Meningiomas of the ventricle system are ex- tered one perioperative death and one severely disabled
tremely rare. We report on a series of 16 intraventricular patient. All other patients had a Glasgow outcome scale
meningiomas (IVMs) treated at our institution between score of 5, and most of the pre-existing symptoms dis-
1980 and 2004, with a special interest on the surgical appeared or improved after surgery. IVMs are a surgically
outcome of using the intra/interparietal and parieto-occip- curable tumour entity in most cases. The intraparietal/
ital approach and the benefits of neuronavigation. A interparietal and parieto-occipital approach is very safe,
retrospective analysis of the medical files for clinicoradio- and neuronavigation allows early devascularisation of the
logical findings, surgical interventions and surgical out- tumour.
come was carried out. In 16 IVM patients with a female/
male ratio of 11:5, age ranged from 24 years to 84 years Keywords Meningioma . Intraventricular . MIB-1 .
(median 44 years). Duration of symptoms ranged from a Surgery . Neuronavigation
few days to several years, and the cardinal symptoms were
signs of increased intracranial pressure (86%), followed by
corticospinal tract signs (43%), visual field defects (36%), Introduction
cognitive changes (29%) and seizures (7%). The majority
of tumours was located in the trigone (88%), and one was Meningiomas are usually benign tumours that are thought
found in each the temporal horn and in the fourth ventricle. to originate from arachnoid cap cells (cells forming the
Tumour size ranged from 2.5 cm to 8 cm (median 5 cm), outer lining of the arachnoid membrane), related cells such
and the radiological appearance was uniform. The neu- as arachnoidal fibroblasts, or perhaps the meningeal
ropathological workup revealed most IVMs as menin- precursor cellthe so-called meningoblast [33]. They ap-
gothelial, transitional (mixed) or lymphoplasmacyte-rich pear predominantly in women, with a female-to-male ratio
meningiomas (81%). Three tumours were classified as of 2:1 [4, 11, 19]. The occurrence in the general population
atypical (19%) and the MIB-1 proliferation index ranged varies from 1.5 to 5.5 per 100 000 inhabitants, and they
from 1% to 40%. Complete resection was possible in all account for 13.4% to 40% of all intracranial tumours,
but one case. The trigonal IVMs were resected via an representing the second most common primary brain
intraparietal/interparietal or parieto-occipital approach, and tumour in adults [4, 6, 18, 32, 34]. The incidence of
neuronavigation was used in eight tumours. We encoun- meningiomas increases with age and demonstrates a peak
occurrence in the sixth and seventh decade [19]. The
A. Bertalanffy (*) . K. Roessler . M. Neuner . E. Knosp distribution of intracranial meningiomas demonstrates the
Neurosurgical Department, Medical University of Vienna, highest incidence at the convexity (35%), followed by
Waehringer Guertel 18-20,
1090 Vienna, Austria parasagittal (20%), sphenoidal (20%), infratentorial (13%),
e-mail: alexander.bertalanffy@meduniwien.ac.at tuberculum sellae (3%) and other locations (4%) [1]. An
Tel.: +43-1-404002566 intraventricular appearance is very rare and ranges from
Fax: +43-1-404004566 1% to 5% of all intracranial meningiomas [1, 2, 4, 6, 9]. A
O. Koperek . E. Gelpi higher incidence is reported in von Recklinghausens
Institute of Neurology, Medical University of Vienna, disease, at 16.6% [5], and in children8% to 11% [11, 21,
Vienna, Austria 35]. Intraventricular meningiomas (IVMs) account for
9.8% to 14% of all intraventricular tumours and for 20% of
D. Prayer
Division of Neuroradiology, Department of Radiology, lateral ventricle tumours [13, 25]. Within the ventricular
Medical University of Vienna, system, they are usually found in the trigone of the lateral
Vienna, Austria ventricle (80%), followed by the third (15%) and fourth
31
Table 1 Presenting signs/symptoms and outcome, excluding two Age ranged from 24 years to 84 years (median 44 years),
patients with incidental findings (n=14) and we observed a female predominance, with a female:
Symptoms Total (%) Improved (%) New male ratio of 2.2:1.
Signs of increased 12 (86%) 12 (100%)
intracranial pressurea Clinical presentation
Corticospinal tract signsb 6 (43%) 5 (83%) 1d
Visual field defect 5 (36%) 3 (60%) 2 In two patients the IVM was an incidental finding. In the
Cognitive changesc 4 (29%) 4 (100%) symptomatic patients, the duration of symptoms ranged
Seizures 1 (7%) 2 from a few days to 7 years (median 6 months). The most
Cranial nerve dysfunction 1d common initial symptoms prior to hospitalisation, such as
a
Headaches, nausea, vomiting and disturbed state of consciousness headaches, nausea, vomiting and disturbed state of con-
b
Hemiparesis and/or hemihypaesthesia sciousness, including one patient who was comatose at the
c
Dysphasia/aphasia, dyslexia/alexia, dysgraphia/agraphia and dys- time of admission, were related to increased intracranial
calculia/acalculia pressure (86%). In addition, we observed sensomotor
d
Meningioma adherent to the bottom of the 4th ventricle (CN V, -XI, deficits (43%), e.g. hemiparesis and/or hemihypaesthesia,
-X palsy, tetraparesis)
visual field deficits (36%) and seizures (7%). The four
largest left-sided IVMs (68 cm diameter) caused cognitive
(5%) ventricles [2, 4, 9, 23, 26]. They are commonly changes such as dysphasia/aphasia, dyslexia/alexia, dys-
believed to arise either from the stroma of the choroid graphia/agraphia and dyscalculia/acalculia (Table 1).
plexus or from the tela choroidea [33].
We report on our series of 16 intraventricular meningiomas,
with a special emphasis on the clinico-radiological appear- Radiological appearance
ance and neuropathological findings. In addition, we discuss
the surgical approach and the benefit of neuronavigation. Tumour size ranged from 2.5 cm to 8 cm (median 5 cm).
Fourteen tumours were located in the trigone of the lateral
ventricle (88%); one was found in each of the temporal
Material and methods horn (6%) and the fourth ventricle (6%). Of 15 lateral
ventricle meningiomas, the side affected was nearly equal,
We performed a retrospective review of patients suffering with 53% right-sided and 47% left-sided tumours.
from IVMs who were treated at the Neurosurgical Depart- On CT and MRI all tumours presented as a well-defined
ment, Vienna Medical School, between 1980 and 2004. The mass. After application of contrast medium, all tumours
medical records, surgical records and imaging studies were displayed a homogeneous strong enhancement. Hydro-
analysed. cephalus or a trapped temporal or occipital horn and peri-
For neuropathological examination, formalin-fixed and tumoral oedema was observed in all but the three smallest
paraffin-embedded tumour specimens of all 16 cases were IVMs. Minimal-to-massive calcifications were observed in
retrieved from the files of the Institute of Neurology, half of the cases, and cystic/necrotic parts within the tu-
Medical University of Vienna, where 35 m-thick sec- mour were absent.
tions were cut for routine histological processing and Angiography was available in four trigonal IVMs and
stained with haematoxylin and eosin. Additionally, immu- showed that the tumours had been supplied by either both
nohistochemical analysis, using anti-Ki67 antibodies (MIB-1, anterior and posterior choroidal arteries or by only pos-
mAb, 1:50, DAKO, Denmark), was performed. For antigen terior choroidal arteries (Table 2).
retrieval, sections were boiled for 20 min in citrate buffer.
As a secondary system we used the ChemMate detection
kit (DAKO). The proliferation index was determined by
immunohistochemical staining of proliferation-associated
nuclear protein Ki67 (MIB-1). Five hundred tumour nuclei Table 2 Neuroradiological features (CT 16, MRI 10, angiography: 4).
were counted by eye; grid and percentage of labelled and AChA Anterior choroidal artery, PChA posterior choroidal artery
unlabelled nuclei were assessed in the tumour regions with
the highest mitotic activity. Neuroradiological feature Number Percentage
The outcome was measured with the Glasgow outcome CT/MRI: clear demarcation 16 100%
scale (GOS) [14]. Homogeneous contrast enhancement 16 100%
Hydrocephalus 14 88%
Peritumoral oedema 13 81%
Results
Calcification 8 50%
Cysts/necrosis 0 0
For the period between 1980 and 2004 we identified 16
Angiography: AChA supply 3 75%
intraventricular meningiomas in our patients files, repre-
PChA supply 4 100%
senting an incidence of 1.5% of all cranial meningiomas.
32
Fig. 1 Coronal MRI scan, T1-
weighted after contrast, demon-
strating a typical trigonal IVM
occupying the cella media and
the temporal horn of the right
lateral ventricle (left). Three
months after complete intra-
parietal resection, no residual
tumour; part of the surgical
corridor (arrow, right)

Histological appearance Interventions and outcome

All tumours showed characteristic features of meningioma. Because of old age and poor medical condition, one patient
On the basis of plain histology, the tumours were classified was treated by endoscopic biopsy and CSF shunting alone.
according to WHO criteria (WHO 2000) as meningothelial In the other 15 patients, complete tumour resection was
meningioma (8/16), transitional (mixed) meningioma (4/16), possible in 14 patients (93%); one resection was incom-
lymphoplasmacyte-rich meningioma (1/16) and atypical plete due to extensive calcifications and adhesion to the
meningioma (3/16). ventricle wall. The fourth-ventricle meningioma was re-
Proliferation indices assessed by immunohistochemical sected via a suboccipital approach; the temporal horn
MIB-1 staining ranged from 1% to 11% but were 13%, meningioma was mimicking a sphenoidal meningioma and
23% and 40% in the atypical meningiomas. was resected via a pterional route. As no tumour attach-

Fig. 2 Snapshot of the Med-


tronic StealthStation during the
resection. Two trajectories are
planned to the choroid plexus of
the temporal horn and the cella
media. The tip of the pointer is
placed at the plexus of the
temporal horn
33

ment to the skull base was found we split the sylvian fissure mass effect, or the surrounding brain oedema. In our series,
and found a tumour between the middle cerebral artery duration of symptoms also varied widely, from a few days
branches originating from the temporal horn. For the 14 to 7 years. The main symptoms we observed were signs of
trigonally located IVMs we used a parietal approach increased intracranial pressure, sensomotor deficits, visual
either through the superior parietal lobule or through the field defects and cognitive defects in the case of large left-
interparietal sulcus or a parieto-occipital approach in all side tumours. Overall, tumour incidence, distribution of
cases (Fig. 1). In the last eight procedures a neuronaviga- patients ages and gender, duration of symptoms and the
tion system was used (Easy-Guide, Philips, Da Best, The symptoms themselves in our series correspond to those in
Netherlands, and StealthStation TREON, Medtronic, previous reports [4, 8, 12].
Minneapolis, Minn., USA) (Fig. 2).
In the early 1980s one patient died from intracerebral
haemorrhage during the postoperative course, and the Radiology
fourth ventricle meningioma was very adherent to the
brainstem, resulting in severe neurological deficit with With CT and MRI, neurosurgeons nowadays possess non-
tetraparesis, bilateral CN V palsy, CN IX and CN X palsy invasive tools to diagnose intraventricular tumours easily.
(GOS 3). Signs of increased intracranial pressure dis- Depending on neuroradiological features in combination
appeared in each patient. Cognitive deficits improved in all with location and age distribution, a high percentage of
(4/4), sensomotor deficits in most (5/6) cases. Even three radiological diagnoses of IVMs can be made by excluding
out of five visual field deficits were ameliorated. As new choroids plexus carcinoma/papilloma, ependymoma, glio-
postoperative symptoms, we observed visual field deficits ma or central neurocytoma [13, 20]. MRI and CT, now-
and seizures in two patients each (Table 1). adays, give us enough information to plan the resection of
an IVM. The surgically important question concerning
displacement of the anterior choroidal artery by the tumour
Discussion can be answered by MRI angiography alone, without the
use of the invasive diagnostic tool of intra-arterial angiog-
In most cases, IVMs are surgically curable tumour entities. raphy and its related risk of complications.
The intraparietal/interparietal and parieto-occipital ap- The radiological appearance is similar to meningiomas
proach can be used very safely in the dominant and non- at other locations. Calcifications are reported to appear in
dominant hemisphere. Neuronavigation systems offer the 47% of all cases; peritumoral oedema can be present [3, 4,
benefits of exact surgical planning and the opportunity to 8, 12, 13, 20, 22]. For our 16 IVMs, angiography was
dissect the tumours blood supply at the beginning of the performed in four patients, CT in 16 patients and MRI in
resection. ten patients, and our findings correspond to those of pre-
The first IVM was reported by Shaw in 1854 [31]. Since vious reports (Table 2).
then, more and more reports on IVMs have been published,
and, in 1986, Criscuolo and Symon identified 400 cases in
the literature [4]. In a recent publication by Nakamura et al. Neuropathology
132 additional cases were supplemented, resulting in a total
of 532 reported IVMs to date [26]. Of these tumours the Owing to their various histological characteristics, menin-
majority was located in the trigone (77.8%), followed by giomas are of great interest to pathologists. Today, we
the third (15.6%) and fourth (6.6%) ventricles. IVMs are distinguish between many histological subtypes [19].
reported to have an incidence of 1% to 5% of all in- Reports about intraventricular meningiomas verified ac-
tracranial meningiomas and comprised 1.5% in our series cording to these currently accepted criteria and reports on
[1, 2, 4, 6, 9]. Patients ages ranged from 24 years to 84 proliferation indices are very rare [4, 8, 12, 26]. In our
years, and we observed a high predominance of women; series the majority of tumours were of the meningothelial
the ratio of women to men was 11:5. or mixed type (75%) and, interestingly, 19% were atypical.
Usually, these tumours reach a large size before patients The MIB-1 proliferation index was high and ranged from
become symptomatic, and diameters of 6 cm and more are 1% to 11% but was 14%, 23%, and 40% in the atypical
not uncommon [8, 12, 15]. Duration of symptoms, from the cases.
first sign to the diagnosis of an intraventricular tumour, is
reported to last from a few months up to 20 years [4, 8, 12].
Over 60 years ago, Cushing and Eisenhardt had already Surgery
summarised these symptoms in their classical publication,
in detail, as lateral ventricle syndrome [6]. Summarizing The deep location, the proximity of motor, sensory, optical,
the literature, one finds that symptoms caused by trigonal and language cortex, as well as the optic radiation and the
tumours are non-specific. The most common symptoms anatomy of the blood supply, make the removal of that
reported are headaches, nausea and vomiting, as well as tumour a neurosurgical challenge. For resection of tri-
visual field defects, speech disturbances, sensomotor def- gonally located meningiomas different approaches were
icits and seizures [4, 8, 12, 22]. These symptoms can be described. The goal of all approaches is to minimise the
easily explained by the typical location in the trigone, their risk of postoperative deficits by using small corticotomies,
34

minimal retraction of surrounding functional brain tissue, Minn., USA), in combination with cylindrical stereotactic
early access and interruption of the tumours blood supply retractors, since 1980. Morita and Kelly described the
and piece-by-piece resection. In 1938, Cushing and efficacy of displaying the tumour, the position of sulci, the
Eisenhardt advocated a temporoparietal approach [6]. route through non-essential brain tissue, and multiplanar
Later, a transfrontal approach, a posterior middle temporal trajectories. Thus, damage to normal brain tissue, and
gyrus approach, a posterior inferior temporal gyrus ap- morbidity, can be reduced. They achieved total tumour
proach, a parieto-occipital approach and a transcallosal resection in all three IVMs. In our institution we have
approach were championed [7, 8, 15, 16, 28]. Nowadays, gained much experience in neuronavigational procedures
the most frequently used approaches are the parieto- during the past decade [29, 30]. The use of these systems is
occipital, the middle temporal gyrus and the trans-splenial of benefit to the patients, as they allow a small skin incision
[23]. The paramedian parieto-occipital approach offers and small craniotomy. In addition, an exact localisation of
good overall access to the tumour but less access to the the entry point, with regard to possible bridging veins, is
tumours blood supply. It can be used in both the dominant possible, and a multiplanar trajectory to the tumour helps
and non-dominant hemisphere, and the risk of direct the surgeon to avoid eloquent structures during approach
damage to the optic radiation is low. The middle temporal and resection. Besides the advantage of gaining space for
gyrus approach offers the advantage of early occlusion of surgery by simple puncture of a trapped temporal or
the tumour-feeding anterior and posterior choroidal artery occipital horn, neuronavigation allows for identification
branches, as well as decompression of a possibly trapped and early dissection of the choroidal arteries. Thus, a
temporal horn, but there is the disadvantage of damage to completely avascular tumour can be easily resected.
the inferior portion of the optic radiation, as well as
language deficits, if such an approach is used in the
dominant hemisphere. The trans-splenial approach causes Conclusion
fewer postoperative seizures than does the transcortical ap-
proach (up to 29%), and speech as well as visual disturbances The clinical presentation and radiological appearance of
should not occur. However, it has the disadvantage of a IVMs are uniform. In most cases they are surgically curable
possible visualverbal disconnection syndrome [2, 4, 810, tumour entities. The intraparietal/interparietal and parieto-
1517, 24]. To decompress a hydrocephalus, preoperative occipital approach is very safe in both hemispheres, and
internal CSF shunting and external ventricular drainage neuronavigation allows early devascularisation of the
remain an alternative [7]. In a case report, Couillard et al. tumour.
took advantage of a combined temporal and parietal
approach, resulting in total tumour resection and excellent
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