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|Year : 2015 | Volume
| Issue : 1 | Page : 32-35
Pediatric meningiomas an aggressive subset: A clinicopathological and immunohistochemical study
M Hui1, MS Uppin1, M Vijaya Saradhi2, BP Sahu2, AK Purohit2, C Sundaram1
1 Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India
2 Department of Neurosurgery, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India
|Date of Submission||30-Jul-2014|
|Date of Decision||25-Aug-2014|
|Date of Acceptance||28-Sep-2014|
|Date of Web Publication||15-Dec-2014|
Dr. C Sundaram
Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Background: Meningiomas are uncommon neoplasms in the pediatric age group and differ in various aspects from their adult counterparts. They account for 0.4-4.6% of all primary brain tumors. Aims: To retrospectively analyze the clinicopathological and immunohistochemical features of pediatric meningiomas. Materials and Methods: Meningiomas in patients under 18 years of age diagnosed between January 2001 to December 2011 were analyzed retrospectively. The hematoxylin and eosin stained sections and Ki 67 labelling index (LI) were reviewed for all the cases Results: The pediatric meningiomas accounted for 1.52% of total meningiomas (15/983). The mean age at presentation was 12 years with male to female ratio of 1.5:1. The presenting symptoms were headache, seizures, and motor deficits. The histology included 9 cases (60%) of atypical meningioma (WHO grade II) followed by 4 cases (26.67%) of WHO grade-I and 2 cases (13.33%) of anaplastic meningioma (WHO grade III). Five cases had a recurrence. Ki67 LI ranged from 0.5% to 1.5% in grade I, 0.5% to 15% in grade II and 13% to 24% in grade III meningiomas. Conclusion: Meningiomas are rare in children and show a male preponderance. There was a higher incidence of atypical and anaplastic meningiomas in pediatric population.
Keywords: Meningioma, pediatric age, atypical meningioma, anaplastic meningioma, Ki 67 labelling index
|How to cite this article:|
Hui M, Uppin M S, Saradhi M V, Sahu B P, Purohit A K, Sundaram C. Pediatric meningiomas an aggressive subset: A clinicopathological and immunohistochemical study. J Postgrad Med 2015;61:32-5
|How to cite this URL:|
Hui M, Uppin M S, Saradhi M V, Sahu B P, Purohit A K, Sundaram C. Pediatric meningiomas an aggressive subset: A clinicopathological and immunohistochemical study. J Postgrad Med [serial online] 2015 [cited 2020 Jul 5];61:32-5. Available from: http://www.jpgmonline.com/text.asp?2015/61/1/32/147035
| :: Introduction|| |
Meningiomas predominantly occur in the fifth decade of life accounting for 13.4% to 27.3% of all primary intracranial tumors. Intracranial meningiomas in children are rare, accounting for 0.4 to 4.6% of all primary brain tumors in the age group of 0-18 years.  They account for 0.9-3.1% of all intracranial meningiomas.  The characteristic features of pediatric meningiomas are different from their adult counterparts. They have more malignant histological features and have a worse clinical outcome. We present in this paper, a retrospective analysis of pediatric meningiomas seen by us over a decade.
| :: Materials and Methods|| |
Ethics, study design, study period: The study period was Jan 2001 to Dec 2011 and the design retrospective. Institutional review board approval was taken and confidentiality maintained. The details including age, gender, clinical presentation, location, preoperative radiological finding and recurrence if any were noted from medical records. The biopsy specimens were processed for routine paraffin sections. The hematoxylin and eosin-stained section were reviewed for all the cases.
Tumor classification: The tumors were classified into three grades according to World Health Organization (WHO) 2007 criteria. The subtypes clear cell, chordoid, papillary and rhabdoid meningiomas were identified. Special stains with periodic acid Schiff (PAS) and Alcian PAS were done wherever necessary. Immunohistochemistry (IHC) with appropriate panel of antibodies, including epithelial membrane antigen (EMA), vimentin, S100, CD34,desmin, myogenin, smooth muscle antigen(SMA), pancytokeratin(PanCK), glial fibrillary acidic protein(GFAP) and Ki67 (Bio Genex, CA, ready to use), was carried out wherever necessary in histological subtyping of meningiomas.
Details of immunohistochemistry: Briefly, sections of 4-5-μm thickness were prepared and mounted on poly-l-lysine-coated slides. The sections were dewaxed in two changes each in xylene and alcohol and stabilized in 0.1 M phosphate buffer saline solution. Antigen retrieval was carried out by autoclaving sections at 121°C for 10 minutes. The endogenous peroxidase activity was quenched by incubation with 3% peroxide in methanol for 10 minutes.Sections were incubated for 1 hour at room temperature with primary antibodies followed by incubation with secondary antibodies for 30 minutes. Antigen-antibody complex was visualized by using diaminobenzidine as chromogen. Sections were counterstained with hematoxylin, dehydrated and mounted. IHC with Ki67 was done in all the cases. At least 1000 nuclei were counted under higher magnification (40× objective) and the average was expressed as percentage. Care was taken not to recount the same areas and foci of necrosis and blood vessels were excluded.
| :: Results|| |
Demographics: A total number of 983 meningiomas were diagnosed during the study period and of these there were 15 cases in the pediatric age group accounting for 1.52% of all the meningiomas. There were nine males and six females (M:F 1.5:1). The age of the patients ranged from 2 to 18 years. A majority of the cases presented in the second decade of life.
Presenting symptoms and sites of involvement: The presenting symptoms included headache, seizures, papilledema, impairment of vision, focal neurological deficits, sensory deficits and cranial nerve palsies. The duration of the symptoms varied from few weeks to a few years. The common sites of involvement were cerebral convexities, cavernous sinus, posterior fossa, intraorbital, lateral ventricle and spinal region. Most of these tumors on MRI showed isointense to hypointense lesions on T1, hyperintense on T2 and homogenously enhanced with contrast. One case showed cystic change.
Tumor grading: As per WHO 2007 criteria, 9/15 tumors were atypical meningiomas (WHO grade II) accounting for 60% of cases. The histological features included hypercellularity, mitosis (more than 4/10 HPF), sheeting of the tumor cells, nuclear pleomorphism, necrosis and brain infiltration [Figure 1]. Among these tumors, two were clear cell meningiomas comprising of sheets and whorls of tumor cells with clear cytoplasm and round vesicular nucleus with prominent nucleoli. The cells showed PAS positivity. Brain invasion was noted in one of these clear cell meningiomas. There was a single case of chordoid meningioma located in the intraspinal region comprising of polygonal cells with clear to acidophilic cytoplasm and prominent nucleoli in a myxoid background which was alcian PAS positive [Figure 2]. The grade 1 meningiomas were predominantly transitional meningiomas accounting for 20% of cases and a single case of fibroblastic meningioma. In transitional meningiomas the tumor cells were arranged in compact whorls and in fibroblastic meningioma the cells were arranged in intersecting fascicles embedded in a collagen rich matrix [Figure 3].
|Figure 1: Transitional meningioma. (a and b) Tumors cells arranged in whorls (Hematoxylin and eosin,×100& x400); (c) Ki67 labeling index 1.5% (HRP-Polymer, × 100)|
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|Figure 2: Atypical meningioma (a) tumor cells arranged in sheets (Hematoxylin and eosin,×100); (b) atypical mitosis (Hematoxylin and eosin,×400);Ki67 labeling index-7%(HRP-Polymer,×100)|
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|Figure 3: Chordoidmeningioma (a) cords and trabeculae of polygonal cells with clear to acidophilic cytoplasm in a myxoid background (Hematoxylin and eosin, ×100); positive staining of tumor cells on IHC with (b) EMA, (c) vimentin, (d) S100 (e) Ki67 labeling index -2% (HRP-Polymer, ×100)|
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There were 2 cases of anaplastic meningioma (WHO grade III) comprising of one papillary and another rhabdoid meningioma. The rhabdoid meningioma showed large polygonal acidophilic cells with eccentrically located round nucleus. These cells were positive for EMA, vimentin and negative for GFAP, desmin, myogenin, SMA and S100 [Figure 4]. The papillary meningioma had papillae lined by cells which were EMA and vimentin positive [Figure 5]. Brain invasion was noted in 5 out of 15 meningiomas. The Ki67 LI ranged from 0.5% to 1.5% in grade I tumors, 0.5-15% in grade II and 13-24% in grade III tumors.
|Figure 4: Rhabdoid meningioma (a) squash smear (Hematoxylin and eosin ×100); (b) sheets of rhabdoid cells (Hematoxylin and eosin ×100); (c) polygonal cells with abundant dense acidophilic cytoplasm and eccentric nucleus (Hematoxylin and eosin ×400); (d) Ki67 labeling index -24%(HRP-Polymer,×100)|
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|Figure 5: Papillary meningioma (a) Papillae with fibrovascular core lined by stratified layers of cuboidal to low columnar cells (Hematoxylin and eosin ×100); (b) positive staining of tumor cells on IHC with EMA (HRP-Polymer×100)|
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Follow up details: Out of 15 cases of pediatric meningiomas, 5 cases had recurrence and were re-operated which included one grade I, two each in grade II and grade III tumors. None of the tumors on recurrence progressed to higher grade as a protocol, all grade II and grade III tumors were sent for radiotherapy. Follow-up details were not available except in recurrent cases. No data was available regarding mortality. The clinicopathological and radiological details have been summarized in [Table 1].
|Table 1: Summary of clinical, radiological and pathological findings of pediatric meningiomas|
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| :: Discussion|| |
Meningiomas are uncommon neoplasms in the pediatric age group. However, recent studies have shown a rising trend of pediatric meningiomas. This may be attributed to improvement in various diagnostic modalities. In our series of pediatric meningiomas the incidence was 1.52%, similar to observation of Mendiratta et al. and Mehta et al., Babuet et al. reported 11 cases of pediatric meningiomas out of 429 cases of meningiomas accounting for 2.56%.  The pediatric meningiomas usually present in later half of first decade or early part of second decade of life. In our case series majority of the cases were in the second decade of life. Infantile meningiomas are extremely rare and in our study we did not have any infantile meningioma. The youngest patient in our series was 2years of age. In several other case series including ours, pediatric meningiomas show a distinct male predominance in contrast to adult meningiomas which have a female preponderance. , This difference reflects the absence of the effect of sex hormones on corticosteroid receptors in meningioma cells and also suggests a different pathogenic mechanism of pediatric meningiomas.  Neurofibromatosis-associated meningiomas occur earlier in life than sporadic meningiomas and may be the presenting feature of neurofibromatosis type II especially in pediatric population. Though most of these are WHO grade I tumors, studies have shown that these tumors have a more aggressive behavior than sporadic meningiomas. Several series have shown close associations of pediatric meningiomas and neurofibromatosis accounting for 41% as compared to 0.35% in adult counterparts.  In our series none of the cases were associated with neurofibromatosis.Seizures were present in 25% of patients reported by Germano et al. and 31% of patients by Merten et al. similar to our series (26.67%) and was much lower than in adult meningiomas. , The unusual localization of meningiomas was seen more frequently in children than adults. Merten et al. and Herz et al. reported intraventricular location of meningiomas in 17% and 44% of cases respectively as compared to 5% in adults. , In our case series there was a single case of intraventricular meningioma. The adult meningiomas are more commonly seen in the cerebral convexities as compared to children. In our study, 8 cases (53.33%) were seen in cerebral convexities. There is a lack of dural attachment in children due to the origin of meningioma in leptomeningeal elements located within the parenchyma or near the ventricles. These tumors can reach large size as the ability of the skull to compensate volume changes is facilitated in this age. 
The histopathology of meningiomas in the first two decades of life has a higher incidence of atypical and anaplastic subtypes (9%) as compared to adult population (2.8%).  Babu et al. in their case series of meningiomas have documented eight cases of atypical (Grade II) meningiomas in the pediatric age group accounting for 72.73% cases as compared to 16.31% of cases in adults.  Germano et al. in their case series documented 71% of meningiomas as grade I, 29% as grade II (atypical) and none were anaplastic.  In our series 60% of the cases were atypical and 13.33% were anaplastic meningiomas. A comparative analysis of pediatric meningiomas has been summarized in [Table 2]. ,,,,,
Papillary meningiomas are more frequently seen in children and are more aggressive than other subtypes of meningiomas.  In our series there was one case of papillary meningioma in a 5 year old child. In the series of Perry et al. a high frequency of brain invasion was noted in pediatric meningiomas.  In our series 5 out of 15 meningiomas had brain invasion. Sandberg et al. documented a higher Ki67 LI in atypical and anaplastic meningiomas as compared to grade I meningiomas similar to our study.  . In conclusion, pediatric meningiomas are uncommon (1.52%) and have a male preponderance with a predilection to occur at unusual sites. Higher grades and aggressive histologic subtypes with higher rates of recurrence are noted in children.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
[Table 1], [Table 2]