<%server.execute "isdev.asp"%> A treatise on pediatric meningiomas: Single-center retrospective cohort experience and review of literature Jain S, Muzumdar D, Shah A, Goel A - J Pediatr Neurosci
home : about us : ahead of print : current issue : archives search instructions : subscriptionLogin 
Users online: 986      Small font sizeDefault font sizeIncrease font size Print this page Email this page

  Table of Contents    
Year : 2020  |  Volume : 15  |  Issue : 3  |  Page : 238-244

A treatise on pediatric meningiomas: Single-center retrospective cohort experience and review of literature

1 Department of Neurosurgery, Topiwala National Medical College & B. Y. L. Nair Charitable Hospital, Mumbai, Maharashtra, India
2 Department of Neurosurgery, Seth Gordhandas Sunderdas Medical College (GSMC) and the King Edward Memorial (KEM) Hospital, Mumbai, Maharashtra, India

Date of Submission19-Aug-2019
Date of Decision04-Nov-2019
Date of Acceptance27-Jan-2020
Date of Web Publication06-Nov-2020

Correspondence Address:
Dr. Sonal Jain
Department of Neurosurgery, Topiwala National Medical College and B. Y. L. Nair Charitable Hospital, Dr. A. L. Nair Road, Mumbai, Maharashtra.
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_102_19

Rights and Permissions



Background: Pediatric meningiomas are infrequently encountered in clinical practice. In comparison to adults, they have a distinct pathophysiology and clinical presentation. They are benign but locally aggressive tumors. Radical excision often culminates in good outcome. Aim: The aim of this study was to study the demographic profile, clinico-radiological features, pathophysiology, and surgical outcome of childhood meningiomas. Materials and Methods: The case records of patients <18 years of age operated for meningiomas in our institute from 1985 to 2015 were retrieved. The demographic profile, clinical and radiological features, surgical approach, extension of resection, and surgical outcome were recorded and subsequently analyzed. Results: Among 37 patients, 20 were males and 17 were females. The mean age was 13 years. Predisposing etiologies such as neurofibromatosis and radiation exposure were identified in 20% of the study population. There were 31 intracranial and 6 spinal meningiomas. Headache associated with vomiting was the most common presenting complaint. Majority were supratentorial tumors. A safe maximal excision was attempted in each case. Recurrence was noted in five patients. Average follow-up was approximately 24 months. Two patients succumbed to the illness. Approximately, 30% of patients were rendered morbid. Conclusion: Although rare, pediatric meningiomas are biologically different from their adult counterparts. They have a male predominance, common in intraventricular region and cystic in nature. Radical excision is associated with good prognosis. Although benign, they are aggressive in nature and have a tendency to recur. The response to adjuvant therapy is modest. Further molecular research and genetic studies are necessary to understand the biology of pediatric meningiomas, which will help in the identification of targeted molecular therapy.

Keywords: Pediatric, meningioma, surgery

How to cite this article:
Jain S, Muzumdar D, Shah A, Goel A. A treatise on pediatric meningiomas: Single-center retrospective cohort experience and review of literature. J Pediatr Neurosci 2020;15:238-44

How to cite this URL:
Jain S, Muzumdar D, Shah A, Goel A. A treatise on pediatric meningiomas: Single-center retrospective cohort experience and review of literature. J Pediatr Neurosci [serial online] 2020 [cited 2022 Dec 6];15:238-44. Available from: https://www.pediatricneurosciences.com/text.asp?2020/15/3/238/300030

   Introduction Top

Meningiomas are second to gliomas in terms of frequency of occurrence in adults. However, pediatric meningioma is a rare entity. They account for 0.4%–4.6% of all primary childhood brain tumors.[1] There is definite role of predisposing factors such as previous radiation exposure and presence of neurofibromatosis (NF) in development of these tumors.[2] There is slight male predominance noted with an average sex ratio of 1.2:1, unlike their adult counterparts.[1] They are generally benign in histology, but locally aggressive. Total radical excision often portends to good outcome in this population.

   Materials and Methods Top

A retrospective analysis of all patients under 18 years of age operated and treated for meningiomas in the King Edward Memorial (KEM) Hospital, Mumbai, Maharashtra, India, since 1985 till 2015 was performed. Information encompassing clinical presentation, radiological features, operative technique, operative finding, and histopathological correlation was documented. Follow-up with emphasis on morbidity and mortality quotients was recorded. A special note of discriminating features from their adult counterparts was made whenever feasible.

Statistical analysis was carried out in Microsoft Excel, Microsoft Corporation, Redmond, WA after due compilation of the data. Measures of central tendency such as mean and median were typically used.

   Results Top

The demographic profile, clinico-radiological features, histopathology, and surgical outcome of pediatric meningioma patients have been studied.

A total of 37 patients formed our study group. The sex ratio was 1:1.2 with 17 females and 20 males. The average age at presentation was 14 years. The age spectrum spanned across from 2.5 to 20 years.

Anatomical distribution

There were six cases of spinal meningiomas and 31 patients of intracranial meningiomas [Figure 1]. There were approximately four cases of orbital meningiomas. There were three children with petroclival meningioma.
Figure 1: Right anterior clinoidal meningioma: (A) preoperative axial T1 CT image postcontrast showing right anterior clinoidal meningioma, (B) preoperative axial T2-weighted magnetic resonance image showing right anterior clinoidal meningioma, and (C) postoperative axial CT image postcontrast showing partial staged resection of the lateral portion of the meningioma

Click here to view

Of the supratentorial tumors, parietal convexity meningiomas formed the majority [Figure 2]. The supratentorial meningiomas have been subclassified based on the anatomical location [Table 1]. There were only two cases of infratentorial meningiomas [Figure 3].
Figure 2: Right frontoparietal meningioma: (A) preoperative axial CT image postcontrast showing right frontoparietal convexity meningioma and (B) postoperative axial CT image postcontrast showing radical resection of the meningioma

Click here to view
Table 1: Anatomical subclassification of supratentorial pediatric meningiomas

Click here to view
Figure 3: Preoperative image of a patient with foramen magnum meningioma: sagittal T1-weighted magnetic resonance image showing foramen magnum meningioma

Click here to view

Clinical presentation

Majority of children presented with signs and symptoms secondary to raised intracranial pressure. The presenting complaints with their relative frequencies have been summarized in [Table 2].
Table 2: Clinical presentation

Click here to view

Predisposing factors

NF was the most frequent inciting etiology and was present in approximately 16% of patients. Radiation exposure as part of treatment for ALL (acute lymphocytic leukemia) was present in one patient.

Radiological features

The majority of patients from 1985 to 1990s have computed tomographic (CT) images.

There were 15 partially cystic meningiomas. Approximately, 10 patients had intraventricular tumors. The heterogenous contrast enhancement and necrosis implying aggressiveness and higher potential for recurrence were noticed in 12 patients.

Surgical details

A complete radical resection was attempted in every patient in our series. Simpson’s grade II was achieved in majority of cases. Preoperative embolization to preemptively decrease the vascularity of the tumors was performed in two patients. The dural attachment was identified and coagulated in every patient. Planned staged surgery was carried out in two patients. There were three patients who were operated outside and had to be reoperated to achieve maximum safe resection.

Approximately, eight patients developed recurrent tumors and had to undergo resurgery. Majority of the meningiomas in our series were histopathologically benign transitional meningiomas.


In the immediate postoperative period, majority of the patients improved in their preoperative symptoms. There were postoperative convulsions in 16 patients; most of which were managed conservatively. There were 10 patients with immediate postoperative limb weakness; majority of whom improved over a week’s time with cerebral decongestants.

Major morbidity in the form of residual postoperative limb weakness was encountered in six patients. Approximately, 80% of these showed significant improvement in power at 6 months of follow-up. Persistent headache was seen in five patients. There was postoperative cranial nerve paresis in three patients with petroclival meningiomas. All of them had undergone multiple surgeries. The facial paresis improved in two of these patients. There was no improvement in the vision of patients with orbital meningiomas.


The mortality rate of our series was 5.4%. Of the two patients who died, one child was a 12-year-old girl who was operated four times for petroclival meningioma, twice at our institute. She had preoperative history of convulsion and right-sided limb weakness. She had left trigeminal and facial nerve paresis. During her third surgery, she developed intraoperative autonomic disturbances and the surgery was abandoned. She went into status epilepticus postoperatively and could not be salvaged. Histopathology was fibroblastic meningioma.

The second pediatric patient who expired was a 2.5-year-old baby girl with left frontal falcine meningioma. She presented with bilateral lower limb weakness for 3 months and bladder incontinence for 1 month. She had major blood loss intraoperatively, which was adequately replaced. In the postoperative period, she developed pulmonary edema and succumbed to the same.


The most frequent histopathological diagnosis in our series was transitional meningioma as shown in 23 of 37 patients. Malignant angiomatous tumor was documented in two patients. The various histopathological classes of meningiomas encountered in our pediatric population with their relative frequencies have been mentioned in [Table 3] and [Figure 2].
Table 3: Histopathological distribution of pediatric meningiomas

Click here to view

   Discussion Top

Pediatric meningiomas are infrequent primary brain tumors. The incidence is estimated to be approximately 0.5%–3% of all pediatric brain tumors.[1],[2],[3] They behave differently as compared to their adult counterparts. The largest series on pediatric meningiomas is from the Netherlands. Thuijs et al.[4] reported 72 patients of pediatric meningiomas in over 35 years of experience. Our series consisted of 37 patients, studied over 29 years.

Adult meningiomas are most commonly encountered in the fifth and sixth decades of life. Most childhood meningiomas are described in the late first and early second decade of life, as reported by Perry and Dehner.[5] Consistent with the aforementioned findings, the mean age of presentation in our study was 14 years.

Our study population comprised 20 males and 17 females. The male-to-female ratio was 1.2:1. The slight male predominance is reflected in many other pediatric meningioma case series.[1],[3],[4] Unlike pediatric meningiomas, female preponderance in adult meningiomas is linked to the interplay of sex hormones on corticosteroid receptors in the arachnoid cap cells.

There are other mechanisms at play in the pathogenesis of pediatric meningiomas. Begnami et al.[6] have confirmed the role of NF2 gene in tumorigenesis of pediatric meningiomas. They reported NF2 gene deletion with decreased merlin protein expression in as many as 72% of the patients. NF was present in approximately 16% of our patients.

We report approximately six cases of spinal meningiomas and 31 cases of intracranial tumors. Thoracic region is the most common location of spinal pediatric tumors as reported by Baumgartner and Sorenson.[7] This is in accordance with our study. Wang et al.[8] studied approximately 10 cases of spinal meningiomas from 2003 to 2012. They reported worse prognosis in children with NF. Approximately, 50% of our patients with spinal meningiomas had NF. There were preoperative motor deficits in two of them. They showed minimal postoperative improvement. We concluded that absence of predisposing factors such as NF translates in better outcome. Santos et al.[9] shared similar opinion.

Infratentorial meningiomas were found in only 5.4% of patients, with the majority being supratentorial tumors. The most frequent tumor was the parietal convexity meningioma. Baumgartner and Sorenson[7] reported an incidence of approximately 83% of supratentorial tumors. The incidence of supratentorial tumors in our series was 78.3%.

The most common complaints in the pediatric age group with meningiomas reflect features of raised intracranial pressure. Headache and vomiting were seen in approximately 70% of children. Maranhão-Filho et al.,[10] in a study on seven patients with pediatric meningiomas, concluded that headache, seizures, and motor deficits were the most frequent complaints on presentation. Pediatric meningiomas are locally aggressive and often found in intraventricular location, which can explain the propensity to block the cerebrospinal fluid pathways earlier, leading to premature development of hydrocephalus and raised intracranial pressure in these patients. This is in accordance with Mehta et al.,[1] who cited similar findings.

Maximal safe resection as advocated in majority of series on pediatric meningiomas was attempted in every patient. Approximately, eight patients had to undergo staged procedures to reach complete maximum safe resection. The intraventricular nature, attachment to the base of the skull, and cystic nature made radical excision difficult in some of these tumors. Dash et al.[11] have described absence of dural tail in intraparenchymal pediatric meningiomas. The dural tail was identified in most of our patients.[12]

The most frequently encountered meningioma in our series was benign transitional meningioma. The benign histopathological nature did not always mean good prognosis. In our series, the patient of malignant angiomatous meningioma who underwent complete resection had a good outcome. On the contrary, a patient with histopathologically benign grade petroclival meningioma succumbed. Secondary to the arduous location of the meningioma, this patient had undergone multiple surgeries, the last one being for recurrence.

There were eight patients who underwent multiple surgeries with documented recurrence in five of them. Majority were patients with skull base meningiomas [Table 4]. The possible explanation due to the skull base location is that they often defy rules of complete resection. Subtotal resection has been repeatedly proven to lead to recurrence and worse prognosis. Burkhardt et al.[13] reported that skull base meningiomas have relatively poor prognosis as they are less likely to yield to maximally safe radical resection. Ravindranath et al.[14] concluded that resurgery often with adjuvant radiotherapy helps in recurrent cases.
Table 4: Stratification of tumor behavior and its management as per location

Click here to view

The single most defining prognostic factor is extent of resection. The location and ability to completely resect the tumor help prognosticate better than the histopathology. This concurs with the findings of Menon et al.[2]

The presence of predisposing etiologies such as NF and radiation exposure leads to worse prognosis. The intraventricular and base of skull location with cystic nature of some of these tumors leads to partial excision, which then translates into eventual recurrence. These are more relevant prognosticating factors as compared to the histological grade.

Menon et al.[2] have scanned data from 1882 to 2009. They have described younger age (<10 years), superficial position, complete resection, and absence of predisposing conditions (such as NF) as indicators of positive outcome. They concluded that the extent of resection predicts outcome better than the histopathological grade. The same can be appreciated in our study, as the lesions that behaved locally and clinically aggressive were in fact benign on histopathology.

Santos et al.[9] have concluded in the same vein that the presence of predisposing situations such as radiation and NF along with the extent of resection affects the prognosis more than the histopathological grade.

The mean follow-up was approximately 24 months. Adjuvant radiation therapy was advocated for patients older than 3 years, with residual lesion, recurrence, or higher histopathological grade. Four patients received radiation. Owing to limited follow-up, we cannot prognosticate for these patients in the long run. We acknowledge that scarce follow-up data are a major limitation of our study.

A brief comparative review of the major series of pediatric meningiomas published till date is presented in [Table 5].
Table 5: Review of literature

Click here to view


Our study pertaining to our single-institute experience revealed data akin to other major contemporary pediatric meningioma series. This reinforces and consolidates our strategy on the management of these patients at our institute. We do not favor multiple stage surgeries where maximum safe radical resection can be performed. We do not practice preoperative shunt insertion. We favor more of operative management rather than wait and watch management with symptomatic treatment, even for dangerous terrains such as skull base and petroclival regions. Our results have been good and at par with other institutes.

   Conclusion Top

Although rare, pediatric meningiomas are biologically different from their adult counterparts. They have a male predominance, common in intraventricular region, and cystic in nature. Radical excision is associated with good prognosis. Although benign, they are aggressive in nature and have a tendency to recur. The response to adjuvant therapy is modest. Further molecular research and genetic studies are necessary to understand the biology of pediatric meningiomas, which will help in the identification of targeted molecular therapy.[20]

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Mehta N, Bhagwati S, Parulekar G Meningiomas in children: A study of 18 cases. J Pediatr Neurosci 2009;4:61-5.  Back to cited text no. 1
Menon G, Nair S, Sudhir J, Rao BR, Mathew A, Bahuleyan B Childhood and adolescent meningiomas: A report of 38 cases and review of literature. Acta Neurochir (Wien) 2009;151:239-44; discussion 244.  Back to cited text no. 2
Ghim TT, Seo JJ, O’Brien M, Meacham L, Crocker I, Krawiecki N Childhood intracranial meningiomas after high-dose irradiation. Cancer 1993;71:4091-5.  Back to cited text no. 3
Thuijs NB, Uitdehaag BM, Van Ouwerkerk WJ, van der Valk P, Vandertop WP, Peerdeman SM Pediatric meningiomas in the Netherlands 1974-2010: A descriptive epidemiological case study. Childs Nerv Syst 2012;28:1009-15.  Back to cited text no. 4
Perry A, Dehner LP Meningeal tumors of childhood and infancy. An update and literature review. Brain Pathol 2003;13:386-408.  Back to cited text no. 5
Begnami MD, Rushing EJ, Santi M, Quezado M Evaluation of NF2 gene deletion in pediatric meningiomas using chromogenic in situ hybridization. Int J Surg Pathol 2007;15: 110-5.  Back to cited text no. 6
Baumgartner JE, Sorenson JM Meningioma in the pediatric population. J Neurooncol 1996;29:223-8.  Back to cited text no. 7
Wang XQ, Zeng XW, Zhang BY, Dou YF, Wu JS, Jiang CC, et al. Spinal meningioma in childhood: Clinical features and treatment. Childs Nerv Syst 2012;28:129-36.  Back to cited text no. 8
Santos MV, Furlanetti L, Valera ET, Brassesco MS, Tone LG, de Oliveira RS Pediatric meningiomas: A single-center experience with 15 consecutive cases and review of the literature. Childs Nerv Syst 2012;28:1887-96.  Back to cited text no. 9
Maranhão-Filho P, Campos JC, Lima MA Intracranial meningiomas in children: Ten-year experience. Pediatr Neurol 2008;39:415-7.  Back to cited text no. 10
Dash C, Pasricha R, Gurjar H, Singh PK, Sharma BS Pediatric intraventricular meningioma: A series of six cases. J Pediatr Neurosci 2016;11:193-6.  Back to cited text no. 11
Agha RA, Borrelli MR, Vella-Baldacchino M, Thavayogan R, Orgill DP; STROCSS Group. The STROCSS statement: Strengthening the reporting of cohort studies in surgery. Int J Surg 2017;46:198-202.  Back to cited text no. 12
Burkhardt JK, Neidert MC, Grotzer MA, Krayenbühl N, Bozinov O Surgical resection of pediatric skull base meningiomas. Childs Nerv Syst 2013;29:83-7.  Back to cited text no. 13
Ravindranath K, Vasudevan MC, Pande A, Symss N Management of pediatric intracranial meningiomas: An analysis of 31 cases and review of literature. Childs Nerv Syst 2013;29:573-82.  Back to cited text no. 14
Hanel RA, Tatsui CE, Araujo JC, Grande CV, Antoniuk A, Gasparetto EL, et al. [Meningiomas in pediatric patients: Report of 2 cases]. Arq Neuropsiquiatr 2001;59:623-7.  Back to cited text no. 15
Alexiou GA, Mpairamidis E, Psarros A, Sfakianos G, Prodromou N Intracranial meningiomas in children: Report of 8 cases. Pediatr Neurosurg 2008;44:373-5.  Back to cited text no. 16
Sheikh BY, Siqueira E, Dayel F Meningioma in children: A report of nine cases and a review of the literature. Surg Neurol 1996;45:328-35.  Back to cited text no. 17
Arivazhagan A, Devi BI, Kolluri SV, Abraham RG, Sampath S, Chandramouli BA Pediatric intracranial meningiomas––Do they differ from their counterparts in adults? Pediatr Neurosurg 2008;44:43-8.  Back to cited text no. 18
Gao X, Zhang R, Mao Y, Wang Y Childhood and juvenile meningiomas. Childs Nerv Syst 2009;25:1571-80.  Back to cited text no. 19
Erdinçler P, Lena G, Sarioğlu AC, Kuday C, Choux M Intracranial meningiomas in children: review of 29 cases. Surg Neurol 1998;49:136-40; discussion 140-1.  Back to cited text no. 20


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5]


Print this article  Email this article
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (1,033 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

    Materials and Me...
    Article Figures
    Article Tables

 Article Access Statistics
    PDF Downloaded47    
    Comments [Add]    

Recommend this journal