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Unformatted text preview: [Downloaded free from http://www.pediatricneurosciences.com on Tuesday, December 01, 2009] Original Article
Meningiomas in children: A study of 18 cases
Nirav Mehta, Sanat Bhagwati, Geeta Parulekar
Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India Address for Correspondence: Dr. S. N. Bhagwati, Room No. 129, 1st Floor, MRC, Bombay Hospital, 12 New Marine Lines,
Mumbai: 400020, Maharashtra, India. ABSTRACT
Background: Intracranial meningiomas are rare tumors in children accounting for 0.4-4.6% of all primary brain
tumors in the age group of 0-18 years. Objective: To retrospectively analyze the epidemiological profile, clinical
features, radiological findings, type of excision, histopathological findings, and overall management profile
of these patients. Materials and Methods: Eighteen consecutive cases of meningioma in patients under 18
years of age admitted and operated at our institute between the years 1974-2005 were included in this study.
Results: The mean age of patient at presentation to our hospital was 12.81 years. The male to female ratio
was 1.57:1. The median preoperative duration of symptoms was 1.2 years. An increased incidence was seen in
patients with neurofibromatosis. Intraventricular and skull base locations were common. Total tumor excision
was achieved in all cases. Conclusion: A higher incidence of atypical and aggressive meningiomas is seen in
children. Children with complete resection and a typical benign histology have a good prognosis.
Key words: Meningioma, childhood meningioma, pediatric neoplasms, neurofibromatosis Introduction
Meningiomas occur most commonly in the fifth decade
of life, accounting for approximately 15-20% of primary
intracranial tumors. Intracranial meningiomas in children and
adolescents are rare tumors. In most large series, the incidence
of meningiomas before the age of 16 years ranges from 0.4 to
4.6% of all primary brain tumors in this age group.[2-7] They
account for 0.9-3.1% of all intracranial meningiomas.[8-10] The
female preponderance found in adult patients is not seen in
children, the reported male-to-female ratio in children being
1.2:1.[1,11] Risk factors for the development of meningiomas
include the diagnosis of neurofibromatosis type two (NF-2) and
a history of radiation therapy, the so-called radiation-induced
meningiomas. Meningiomas in children have been considered
by some to be more aggressive than their adult counterparts.[13-16] Materials and Methods
Eighteen consecutive cases of meningioma in patients under
18 years of age admitted and operated at Bombay hospital
institute of medical sciences, Mumbai, India, by senior author
(Dr. SNB) between the years 1974-2005 were included in this DOI: 10.4103/1817-1745.57322 Online full text at
http://www.pediatricneurosciences.com study. All cases were confirmed by radiological, operative, and
histopathological findings. We have tried to retrospectively
analyze the epidemiological profile, clinical features,
radiological findings, type of excision, histopathological
findings, and overall management profile of these patients.
All patients/relatives were called for the follow-up or an
attempt was made to at least get an interview on telephone.
As and where possible, we have made an attempt to determine
the differentiating features between adult and pediatric
We operated a total of 934 meningioma cases between the
years 1974-2005, of which 18 cases were in the age group
of 0-18 years. Thus, childhood meningiomas accounted for
1.92% of total meningiomas in our study.
Sex and Age
Age at diagnosis varied from 9 months to 18 years. We had
only one child with presentation under 1 year of age. The
maximum incidence of meningiomas was seen in the second
decade of life. Thirteen out of 18 cases presented in the second
decade. The mean age at presentation was 12.81 years. There
were 11 males and 7 females in our series. The male-to-female
ratio was 1.57:1.
2009 / Jul-Dec / Volume 4 / J Pediatr Neurosci / 61 [Downloaded free from http://www.pediatricneurosciences.com on Tuesday, December 01, 2009]
Mehta, et al.: Meningiomas in children Presenting symptoms and signs
The most common presenting signs and symptoms were due
to raised intracranial pressure or seizure. The main presenting
symptoms were seizure (8 patients), headache (6 patients),
impairment of vision (2 patients), vomiting (3 patients),
proptosis (2 patients), increased head size (1 patient), and
occipital swelling (1 patient). The median pre-operative
duration of symptoms was 1.2 years.
The most common clinical sign seen was papilledema
(7 patients) followed by monoparesis (4 patients), marked
impairment of vision (2 patients), proptosis (2 patients), tense
anterior fontanelle (1 patient), occipital swelling (1 patient),
and neurofibromatosis (2 patients).
Our series includes patients operated between the years 1974
and 2005. Hence, patients were investigated with varied
available neuroradiological modalities such as plain X-ray of
skull (1 patient with calcified tumor), cerebral angiography
(2 patients), CT scan (11 patients), MRI (2 patients), and
CT scan + MRI (2 patients).
Seventeen cases were supratentorial and 1 case was
infratentorial in location. The tumors were located in the
cerebral convexity in six patients, intraventricular in four
patients, skull base in three patients, Falcine in two cases,
parasellar in one patient, tentorial in one case and in posterior
fossa in one patient.
Tumor was homogenous in appearance in most of the cases
visualized with CT/MRI. Intra-tumoral calcification was seen
in two cases. Intra-tumoral cystic changes were seen in two
cases. Hyperostosis was seen in three cases.
Total excision of tumor was achieved in all cases. In 14 patients,
total excision was achieved in one stage. Three patients required
two-stage surgeries for total excision and one patient required
three-stage surgeries to achieve complete excision.
One patient expired postoperatively due to brainstem
disturbances. He turned out to have malignant meningioma on
histopathology. The mean peri-operative mortality was 5.5%.
Histopathology showed fibroblastic meningioma in three
patients, meningothelial in one patient, transitional in
eight patients, angioblastic in three patients, sarcomatous in
one patient, aggressive syncitial in one patient, and malignant
meningioma in one patient.
62 / J Pediatr Neurosci / Volume 4 / Jul-Dec / 2009 Adjuvant therapy
Postoperative adjuvant radiotherapy was given to four
patients; three patients with histopathology suggestive of
angioblastic meningioma and one patient with aggressive
syncitial meningioma. One patient with sarcomatous
meningioma could not be given radiation, as the age at
diagnosis was only 9 months. However, patient had no
recurrence even at 14 years of follow-up.
Follow up and recurrence
An attempt was made to get follow-up of all patients. The period
of follow-up ranged from 1 to 26 years with a mean follow-up
of 6.1 years. We documented two recurrences in our series on
follow-up. The histopathology in patients with recurrence was
aggressive syncytial in one patient and transitional in the other
patient. Both the patients had a total excision during their
first surgery. The patient with transitional meningioma had
a falcine meningioma and may be some microscopic remnant
must have been left behind, as falx was not excised. During his
redo surgery, we achieved a total excision of falx. In a patient
with aggressive syncytial meningioma, recurrence was seen
after 2 years and in a patient with transitional meningioma,
recurrence was seen after 5 years [Figure 1,2]. Discussion
Meningiomas are uncommon neoplasms in the pediatric age
group and differ in various clinical and biological aspects from
meningiomas in the adult population. It was reported that
the frequency was less than 5% of all pediatric brain tumors.
Its incidence as reported by Mendiratta et al. is 1.5% of
total meningiomas seen in the population. In our series of
childhood meningiomas, i.e. meningiomas in children under
18 years of age, the incidence was 1.92%.
Children with meningiomas present late in the first decade
or early in the second decade of life.[10,11,18,19] In our series,
the mean age at presentation was 12.81 years. Infantile
meningioma is extremely rare. Less than 30 cases of
meningiomas in infants less than 12 months of age have been
reported.[10,19,20] The incidence of infantile meningiomas in
different series of childhood meningiomas varies from 2.4%
to 6.9%[8-10] In our series, only one case (5.5%) of infantile
meningioma was seen.
In contrast to adult meningiomas where a female preponderance
is seen, childhood meningiomas showed a distinct male
predominance.[8-10,12,17,22] In our series, there were 11 males and
7 females. The male-to-female ratio was 1.57:1. The greater
occurrence of meningiomas in males could be related to an
absence of the effect of sex hormones on corticosteroid receptors
in meningioma cells for low blood concentrations.[23-25] This
suggests that different pathogenic factors might account for
the occurrence of meningiomas in children and adults. Some
studies on genetic aberrations in meningiomas in children show
no differences from meningiomas in adults. [Downloaded free from http://www.pediatricneurosciences.com on Tuesday, December 01, 2009]
Mehta, et al.: Meningiomas in children Signs and symptoms related to raised intracranial pressure
are most common in childhood meningiomas. Meningiomas
in children grow faster and occur more frequently in the
ventricles than those in adults; therefore, cerebrospinal fluid
circulation can be easily obstructed in the early stage, which
results in increased intracranial pressure. The incidence of
seizure in childhood meningiomas(25%)[8-10] is lower than that
in adult meningiomas (29-40%). In our series, seizure was
seen in 44.4% of cases (eight cases), i.e., an incidence similar
to adult patients. Focal seizure occurs more commonly in
adults, whereas generalized seizure occurs more in children.
In our series, six patients presented with generalized seizures
and only two patients presented with focal seizure.
In different series in the literature, 0-41% of childhood
meningiomas are associated with neurofibromatosis,[8-10] while
the Figure is only 0.35% for adult meningiomas. In Erdincler
et al. series, 41% of childhood meningiomas were associated
with multiple neurofibromatosis, of which 58% were NF-1
and 42% NF-2. In our series, we had one case each with NF-1
and NF-2, i.e. an incidence of 11.11%.
The frequency of intraventricular meningiomas is very
high (12%) as compared to 0.5-4.5% in adults.[27,28] The
propensity for growth into the ventricular system is explained
by the inclusion of arachnoid cells in the choroid plexus and
In our series, 22.22% of cases had an intraventricular
meningioma. The lack of dural attachment is another
frequent occurrence in pediatric meningiomas (28.5%),
whereas it is extremely rare in adult patients.[13,15] This lack of
dural attachment is probably due to derivation of the tumor
from leptomeningeal elements lodging within the parenchyma
or in or near the ventricles rather than from the dura mater.
An important feature distinguishing childhood meningiomas
from adult meningiomas is their peculiar location. Convexity
meningiomas are most common in adult meningiomas, while
childhood meningiomas have other peculiar location that
increases complexity in their management.[11,32] In our series of 18 cases, only 6 cases were having convexity meningiomas, 4 skull
base, 4 intraventricular, 1 tentorial, 1 posterior fossa, 2 falcine
and one case was parasellar in location. In children, meningiomas
have also been rarely reported to occur in the third ventricle
intraparenchymally and in sylvian fissure.
Various series in the literature have also documented a high
incidence of cystic changes in meningiomas in children.[11,36]
In our series, we had two patients with cystic changes in
meningiomas. In series of Tufan et al., 4 out of 11 meningiomas
showed cystic changes.
Surgical excision has been the treatment of choice for these
tumors. Surgical management poses a formidable challenge
considering their peculiar location, larger size at presentation,
relatively less blood volume in children, and the risks of
prolonged surgery like hypothermia, massive blood transfusion,
etc. Nevertheless, with progress in microneurosurgical and
anesthesiological techniques and considering the benign
nature of disease, surgical excision remains the modality of
choice. Also in children with residual tumor or regrowth, the
risk of radiation to the developing brain favors redo surgery
over radiation therapy. Pre-operative embolization is not
routinely practised in children and there are no conclusive
data on its benefits in children.[37,38] However, the morbidity
of the procedure due to small caliber of vessels in children
should be kept in mind. In our series, pre-operative Digital
subtraction angiography (DSA) with tumor embolization was
done only in one case with skull base meningioma [Figure 3].
In our series, surgical excision was done in all cases. Gross
total excision was achieved in 14 cases (77.78%) in the first
attempt. In three patients, the biopsy was done first followed
by a second stage complete excision. In one patient, complete
excision was done in three stages, i.e. biopsy followed by partial
excision followed by complete excision in third stage. We had
only one patient with 5.5% peri-operative mortality rate.
Various studies in the literature between the years 1970 and
2006 have shown a peri-operative mortality rate of 0.3-10%.
Various interventions have been advised in the literature to
reduce the peri-operative mortality like pre-operative CSF Figure 1: MRI Brain with Gadolinium: Axial view showing intensely enhancing falcine meningioma 2009 / Jul-Dec / Volume 4 / J Pediatr Neurosci / 63 [Downloaded free from http://www.pediatricneurosciences.com on Tuesday, December 01, 2009]
Mehta, et al.: Meningiomas in children Figure 3: MRI brain: Axial view showing skull base meningioma
Figure 2: MRI brain with gadolinium: Coronal view showing the same falcine
meningioma diversion, anti-edema measures, anti-epileptic prophylaxis,
use of postoperative ventilation, etc.
Histopathological examination revealed fibroblastic
meningioma in three cases, meningothelial meningioma in
one, eight were transitional, three were angioblastic, one was
sarcomatous, one was aggressive syncytial meningioma, and
one case was malignant type. Histopathology of these tumors
in the pediatric population varied from those in the adult
population. Overall, most series have shown a high incidence
of atypical and anaplastic meningiomas in children as
compared to the adult population. In our series, six patients
(33.33%) had an atypical or anaplastic histopathology. In the
literature, adjuvant therapy has been advised in these patients
in the form of radiation therapy. In series of Perry et al.,
they noted a high frequency of brain invasion in pediatric
meningiomas and reported them to be phenotypically and
genotypically aggressive as compared to the tumors in
adults. Immunohistochemical proliferative markers have
been studied by Sandberg et al. They documented higher
MIB-1 LI in atypical or malignant tumors as compared to
tumors without atypia. On the other hand, median MIB-1
for pediatric meningiomas without histopathological atypia
did not differ significantly from that for adult meningiomas
In our cases with atypical and aggressive histopathologies, four
were given adjuvant radiotherapy. One patient with malignant
meningioma expired postoperatively and one patient with
sarcomatous meningioma could not be given radiation, as
age at the time of surgery was only 9 months. This patient
was followed up closely and has not shown any recurrence
even after 14 years.
We documented two recurrences in our series during the
follow-up. The period of follow-up ranged from 1 to 26 years
with a mean follow-up of 6.1 years. Histopathology in patients
with recurrence showed aggressive syncytial in one case and
transitional in the other case.
64 / J Pediatr Neurosci / Volume 4 / Jul-Dec / 2009 Different series in the literature have shown a recurrence
rate of approximately 13%. Recurrence seems to be strictly
related to incomplete resection and/or histologic subtype of
the meningioma. Atypical, aggressive, and meningiomas with
cortical invasion show a higher rate of recurrence.
Overall, 16 patients improved neurologically postoperatively,
one remained static, and one patient died postoperatively. Conclusion
Meningiomas in children are rare tumors and account for
1.92% of total meningiomas. Meningiomas in children show
some characteristic differences when compared with their
adult counterparts. These include slight preponderance
in male subjects, higher incidence of intraventricular
and skull base location, and frequent cystic changes.
Pediatric meningiomas tend to present with features of raised
intracranial pressure and seizure. An increased incidence
of meningiomas is seen in patients with neurofibromatosis.
Total surgical excision should be performed wherever
feasible, even if it requires staged resection. Advances in
microneurosurgical and anesthesiological management have
considerably reduced the operative morbidity and mortality.
There is a higher incidence of atypical and aggressive
histological subtypes in the pediatric population. Children
with complete resection of meningioma and a typical benign
histology have a good prognosis like their adult counterparts. References
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Source of Support: Nil. Conﬂict of Interest: None declared. 2009 / Jul-Dec / Volume 4 / J Pediatr Neurosci / 65 ...
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