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Case Report

Pediatr Neurosurg 2012;48:3034 Received: April 10, 2012


Accepted after revision: June 11, 2012
DOI: 10.1159/000341176
Published online: August 21, 2012

Intraventricular Meningioma:
Case Report and Literature Review
Humphrey Okechi a A. Leland Albright a, b
a
Kijabe Hospital, Kijabe, Kenya; b University of Wisconsin Health Center, Madison, Wisc., USA

Key Words lar meningioma that was completely resected, and review
Pediatric meningioma Radiological characteristics the radiological and pathological features and outcomes
Surgery Neuropathology Adjuvant therapy of pediatric intraventricular meningiomas.

Abstract Case Presentation


In this article we describe the clinical course of a child with an
intraventricular meningioma and review the relevant litera- A 7-year-old right-handed girl presented with headache and
ture. A 7-year-old girl with a several-month history of head- progressive right hemiparesis for 3 months. Her headache was
primarily nocturnal and was accompanied by nausea and vomit-
aches had a CT scan that demonstrated a large, enhancing, ing which improved the headache. She had no history of seizures
intraventricular tumor. The tumor was completely excised via but a decline in school performance had been noted in the previ-
a superior parietal approach. Pathological examination re- ous 7 months. On physical examination, she was healthy and alert
vealed a pleomorphic meningioma. Postoperative scans but unable to stand without support. She was macrocephalic, with
demonstrated no residual tumor. Pediatric intraventricular an abnormal cranial percussion sound. She had a right central
facial nerve paresis and a right hemiparesis, with exaggerated
tumors are rare. The surgical objective is complete resection, deep tendon reflexes at the ankle joint but no clonus. Hoffmanns
which appears to be associated with excellent outcomes. sign was absent bilaterally.
Copyright 2012 S. Karger AG, Basel Her brain MRI scan revealed a large, isodense, intraventricu-
lar mass in the atrium of the left lateral ventricle. On T1-weighted
images the mass enhanced homogeneously (fig. 13).
The mass was completely excised via a left parietooccipital
Introduction craniotomy with no postoperative neurological deficits. The his-
topathological diagnosis of the mass was a transitional-type me-
Meningiomas are rare in children and intraventricu- ningioma (fig. 4, 5). Postoperatively she developed aseptic menin-
lar meningiomas are even rarer. In a review of 764 pedi- gitis and a subdural effusion that was initially treated success-
atric tumors conducted by the International Society for fully with a subdural drain. The effusion recurred after 3 months
and resolved permanently after a second subdural drain was in-
Pediatric Neurosurgery, 2% of the tumors were menin- serted for several days (fig. 6). At her last follow-up, 6 months
giomas and, of those, only 10% were intraventricular [1]. postoperatively, she was asymptomatic and her right hemiparesis
We report herein a 7-year-old girl with an intraventricu- had completely resolved.

2012 S. Karger AG, Basel Humphrey Okechi, MD


10162291/12/04810030$38.00/0 Kijabe Hospital
Fax +41 61 306 12 34 PO Box 20
E-Mail karger@karger.ch Accessible online at: Kijabe 00220 (Kenya)
www.karger.com www.karger.com/pne E-Mail humphreyokechi@yahoo.com
Fig. 1. Noncontrast-enhanced lateral T1
MRI.

Fig. 2. Contrast-enhanced axial T1 MRI.

Intraventricular Meningioma: Pediatr Neurosurg 2012;48:3034 31


Case Report and Literature Review
Fig. 3. Contrast-enhanced coronal T1
MRI.
Color version available online

Color version available online


Fig. 4. Low-power magnification showing histopathological fea- Fig. 5. High-power magnification showing histopathological fea-
tures of the transitional type meningioma, with characteristic tures of the transitional type meningioma, with characteristic
whorls. whorls.

Discussion the development of pediatric meningiomas: neurofibro-


matosis and prior cranial irradiation. The child in our
Pediatric meningiomas are unquestionably rare tu- case report had neither neurofibromatosis nor prior ir-
mors, comprising about 2% of pediatric brain tumors in radiation. The frequency of concomitant neurofibroma-
many series 1.6% of 1,294 in the series reported by Mal- tosis in children with meningiomas ranges from 19 to
lucci et al. [1] and 1.9% of 934 meningiomas reported by 29% [1, 3, 4]. A congenital intraventricular meningioma
Mehta et al. [2]. Two factors are known to predispose to associated with NF2 has also been reported [5].

32 Pediatr Neurosurg 2012;48:3034 Okechi /Albright



Fig. 6. Contrast-enhanced postoperative
axial CT scan.

The frequency of meningiomas is increased after radiation, lateral and inferior to the atrium. Because the
childhood irradiation but the latency period is long. Ba- tumors are typically large, their removal is often followed
nerjee et al. [6] reviewed MR scans of 49 adults who re- by the development of ipsilateral subdural hygromas,
ceived radiation for childhood leukemia when they were which may need to be drained or shunted. Outcomes of
18 years old. Eleven of the 49 (22%) developed menin- complete excision are not known and subtotal excisions
giomas 1434 years later (mean 25 years). are associated with worse prognosis [1, 7].
In most series of pediatric meningiomas, the tumors Intraventricular meningiomas arise from arachnoid
occur within the ventricles much more commonly than cells contained within the choroid plexus, the tela choroi-
in adults. In a series of 675 adult meningiomas reported dea or the velum interpositum. In a series of 51 cases of
by Liu et al. [7], only 3.7% were intraventricular. In 4 pe- pediatric meningiomas from the Mayo clinic, ranging in
diatric series of 98 combined cases, 19 (19.4%) were intra- age from 7 to 20 years, 5 histological types were seen: me-
ventricular [1, 2, 7, 8]. Ney et al. [9] reported an intraven- ningotheliomatous, fibrous, transitional, psammoma-
tricular meningioma that developed 23 years after cra- tous, and papillary [10]. In the series of 18 cases from
nial irradiation for acute lymphoblastic leukemia. Mehta et al. [2], the frequency of atypical and aggressive
Children with intraventricular tumors typically pres- meningiomas was higher than in adults, with 8 transi-
ent with generalized symptoms such as headache, leth- tional tumors, 1 sarcomatous, 1 aggressive syncitial, and
argy and vomiting and without lateralizing signs. Intra- 1 malignamt tumor. In the study by Mallucci et al. [1], 5
ventricular meningiomas are typically large noninfiltrat- of 21 pediatric meningiomas were malignant. Byard et al.
ing tumors that enhance homogeneously and contain [11] reported two 9-year-old children with intraventricu-
calcifications in 32% of cases [3]. The majority occur in lar meningiomas; 1 was fibroblastic/angioblastic and the
the trigone of the lateral ventricle. The differential diag- other fibroblastic. The intraventricular meningioma re-
nosis includes choroid plexus papillomas, ependymomas, ported by Curiea et al. [12] had atypical features with ar-
primitive neuroectodermal tumors, teratomas and astro- eas of nectosis and a high nuclear:cytoplasmic ratio, with
cytomas. 15% of cells being positive for Ki 67. The intraventricular
Complete surgical resection is the preferred treatment. meningioma in the report by Orakdogen et al. [13] was
Most intraventricular meningiomas have no dural at- transitional. A metaplastic intraventricular meningioma
tachment. The surgical trajectory to tumors within the has also been reported in a child [14].
atrium requires consideration of the location of the optic

Intraventricular Meningioma: Pediatr Neurosurg 2012;48:3034 33


Case Report and Literature Review
In the Mayo clinic series of pediatric meningiomas, lapse-free survival and overall survival were significantly
the 15-year survival rate was 68% and survival correlated better for those who had gross total resections than for
with pathology, with papillary tumors having the lowest those with subtotal resections, and upfront radiotherapy
survival [10]. Outcomes were not reported separately for was found to be of no benefit [16].
intraventricular meningiomas.
Adjuvant radiotherapy has been used following subto-
tal resection of pediatric meningiomas, with generally Conclusion
poor outcomes. In a series of 34 pediatric and adolescent
patients with intracranial meningimomas, adjuvant ra- Pediatric intraventricular meningiomas are rare tu-
diotherapy was given to patients with residual, malig- mors with characteristic features on MR scans and histo-
nant, and recurrent tumors, with unclear benefit [15]. In pathological features different from their adult counter-
a meta-analysis of 35 series in which 547 children and parts. Gross total resection is the preferred treatment and
adolescents were eligible for overall survival analysis, re- appears to be associated with excellent outcomes.

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34 Pediatr Neurosurg 2012;48:3034 Okechi /Albright



Copyright: S. Karger AG, Basel 2012. Reproduced with the permission of S. Karger AG, Basel. Further
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