Introduction: Pediatric meningiomas (PMs) are rare tumors; they differ from their adult counterparts by their atypicality of location, higher rates of malignant change, male preponderance, recurrence, and sometimes, their association with neurofibromatosis. This case series analyzes the clinical behavior, pathological presentation, location, and its association with neurofibromatosis type 2 (NF2). Methods: This case series consists of pediatric patients between the ages of 4 and 16 years who were hospitalized in the neurosurgical department of our hospital from 2012 to 2021 with different neurological symptoms and a literature review using the PubMed/MEDLINE database. Results: Sixty percent of the patients were males, while 40% were females. The most common neurological manifestations were signs of increased intracranial pressure. NF2 was absent in all patients. The predominant histopathology subtypes are atypical and WHO grade II, representing 30% and 40%, respectively. Conclusion: This study supports the relationship between NF2 and pediatric cerebral meningioma but at a lower concomitant rate from 0 to 13%, taking into consideration our original data and the literature review, contrasting some reported cases, which suggest rates as high as 33%, 50%, and 100% in a very small number of patients. Gross total resection without postoperative radiation therapy for nonmalignant and non-NF2-associated PM proved to be a sufficient and a good treatment option.

Meningiomas are the second most common primary tumors of the central nervous system that occur in adults but are uncommon in children and adolescents and represent 1–2% of intracranial tumors in children [1, 2]. Pediatric meningiomas (PMs) differ from their adult counterparts by their atypicality of location, higher rates of malignant change, and recurrence. Intraventricular meningiomas arise from arachnoid cap cells embedded in the choroid plexus or from the tela choroidea and do not present any dural attachments [3‒5]. Although intraventricular meningiomas are relatively frequent in pediatric patients, they are extremely rare in the third ventricle [6]. Studies have shown male predominance [1, 7‒9]. For supratentorial meningiomas, the most common presenting signs and symptoms are raised intracranial pressure (headache, vomiting, and papilledema) and seizures. While, for infratentorial meningiomas, the most frequent signs are defects of the cranial nerves [1, 10, 11].

Pediatric cerebral meningiomas are associated with neurofibromatosis type 2 (NF2), and some small series have reported occurrences as high as 50% [6] and 33% [7]. Cases of PM that occur after oncological treatment during childhood with several types of radiation-induced tumors have been reported in pediatric patients. Meningioma is the second most common radiation-induced tumor in children [12, 13]. Radiation-associated meningiomas are often aggressive and likely to be multiple; they also have a high recurrence rate [14]. The computed tomography appearance of PM is variable; PMs show a high rate of cyst formation [15, 16]. Magnetic resonance imaging (MRI) of PMs usually shows hypo-to iso-intense patterns on T1- and T2-weighted images [17, 18]. Normally, the enhanced imaging of MRI reveals obviously homogeneous or heterogeneous enhancement [19]. The main treatment for meningioma is total resection, although a conservative approach may be acceptable in small asymptomatic patients [20]. Radiation therapy is recommended for nonresectable tumors, high grade or recurrent tumors [21].

From 2012 to 2021, 10 patients (Table 1) with intracranial meningiomas were selected with the help of patient records containing the clinical history, radiological images, and surgical and pathological data from the hospital’s computerized system. In addition, a literature review was carried out to analyze all publications on PM. The PubMed/MEDLINE database was searched and relevant articles were selected for the review.

Table 1.

Data of patients

 Data of patients
 Data of patients

Patient Data

Ages of patients ranged between 4 and 16 years with a mean age of 9.3 years and a slight male predominance. Of all 10 patients, 60% of the patients were males, while 40% were females. Clinical assessment, specifically for cutaneous features of NF2, was done for all patients. All patients underwent preoperative and postoperative craniocerebral MRI and computed tomography. Figures 1and 2 shows preoperative MRI images of 2 patients. The most common neurological manifestations were signs of increased intracranial pressure (headache). One patient presented with a facial nerve deficit. Supratentorial meningiomas were 8 while infratentorial were 2, representing 80% and 20%, respectively. No patient had a previous history of radiation/chemotherapy for any oncological disease.

Fig. 1.

MRI images (a) and (b). a, b (Patient 1) MRI T1 and contrast showing a parietal papillary meningioma measuring (6.0 × 5.3 cm).

Fig. 1.

MRI images (a) and (b). a, b (Patient 1) MRI T1 and contrast showing a parietal papillary meningioma measuring (6.0 × 5.3 cm).

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Fig. 2.

MRI images (a) and (b). a, b (Patient 7) MRI T2 and contrast showing a malignant left temporal lobe meningioma measuring (7.0 × 6.5 cm).

Fig. 2.

MRI images (a) and (b). a, b (Patient 7) MRI T2 and contrast showing a malignant left temporal lobe meningioma measuring (7.0 × 6.5 cm).

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Surgical Treatment

The Simpson grading system was used to define the extent of tumor resection (total resection: grades 1 and 2, subtotal resection: grade 3, and partial resection: grade 4). All 10 patients underwent only total microsurgical excision of tumors. Lack of dural attachment was also observed in 5 patients, representing 50% of all patients during surgery, and all these were intraventricular meningiomas. None of the patients underwent postoperative radiation or chemotherapy treatment due to the extent of resection.

Post Operatory Results

One patient who had malignant meningioma died 3 weeks after discharge from the hospital. Nine patients survived postoperatively, out of which 7 had no neurological symptoms or deficit. None of the 9 surviving patients presented recurrence of the tumor. Only 2 patients presented mild hemiplegia (muscle strength grade 4) 3 and 4 years postoperatively, respectively.

Follow-Up

Follow-up ranged between 6 months and 9 years for all the 9 surviving patients with a mean follow-up period of 3.2 years. Patients underwent periodic physical examinations and MRI scans for postoperative evaluation of recurrence of tumor and neurological function status.

Histopathological Features

Classification of the histopathological subtypes was performed using the 2007 WHO criteria. The histopathological findings showed a heterogenous pattern, although one type predominated. Six histopathological subtypes were observed in this series namely papillary, atypical, transitional, meningothelial, malignant, and fibroblastic (Table 1). Some histopathological subtypes are shown (Fig. 3). There was a predominance of atypical meningioma, representing 30% of all patients. WHO grade II accounted for 40% of this series. Genetic testing for NF2 with tumor samples was done, and NF2 was not present in any patient.

Fig. 3.

Some histopathological subtypes. Images showing some histopathological subtypes. a Right cerebellum papillary meningioma, WHO grade III. b Right parasellar meningothelial meningioma, WHO grade I. c Third ventricle atypical, WHO II. d Right lateral Ventricle transitional, WHO I.

Fig. 3.

Some histopathological subtypes. Images showing some histopathological subtypes. a Right cerebellum papillary meningioma, WHO grade III. b Right parasellar meningothelial meningioma, WHO grade I. c Third ventricle atypical, WHO II. d Right lateral Ventricle transitional, WHO I.

Close modal

The ratio of male to female sex observed in this study was slightly higher with a 40:60 per cent ratio; hence, a clear sex predominance in our population was not observed, although various other studies have suggested meningiomas in children are more frequent in boys. In the adult population, the incidence of meningioma in females is higher than in males; it is known that several hormones (especially progesterone) may modulate the growth of a large percentage of meningiomas. The difference in sex distribution between adult and PMs might be caused by the different sex hormone levels between the two populations [22‒24].

As observed in other series (Table 2), the commonest signs at presentation were headache, most probably related to increased intracranial pressure due to the rapid increase in volume of tumor. The majority of the tumors in this series are large, which explains symptoms resulting from high ICP due to mass effect.

Table 2.

Literature review of available data [1‒15, 17‒21, 25‒35]

 Literature review of available data [1‒15, 17‒21, 25‒35]
 Literature review of available data [1‒15, 17‒21, 25‒35]

Ventricular meningioma accounted for 50% of the total number in this study (lateral ventricle 40% and third ventricle 10%). The incidence of intraventricular meningiomas tends to be higher than that of adult meningiomas. In one series with 675 adult meningioma patients, intraventricular meningiomas comprised only 3.7% [36].

No recurrence of the meningioma was recorded in all 9 surviving patients, suggesting that the treatment method with gross total resection coupled with the type of histological subtypes may have played a role. Surgical resection was needed due to the risk of progressive enlargement and/or the presence of neurological symptoms. The only patient who died was with a malignant histopathological subtype. Kotecha et al. reported that at the time of diagnosis, the histological subtype of the meningioma impinges on further treatment and future prognosis [37]. Although atypical meningioma represented 30% of all patients in this series, it is important to highlight the variations or heterogeneity of this series, taking into consideration that this study has a relatively small number of patients.

None of all 10 patients in this study had a previous history of radiation therapy, although various studies have proved a strong association between previous radiation therapies for oncological diseases and PM. Other studies have confirmed that radiation-induced meningiomas have a greater biological aggressiveness if compared with sporadic lesions [16], which explains why in this case series, 9 out of 10 patients who presented nonaggressive meningiomas survived and maintained good prognosis.

Studies have shown that PM is often associated with NF2 and other medical conditions, which make them differ from their adult counterparts and may explain why the biological behavior and therapeutic guidelines of NF2-associated meningiomas remain uncertain [21].

In this literature review, 13 PM patients from 7 authors (Table 3), representing 13% of a total of 99 patients from 16 out of 32 authors who provided information about the history of NF2, suggest a lower concomitant occurrence rate of NF2 in pediatric patients with cerebral meningioma as compared to some single research series such as by Santos et al. [7] with 9 patients, Muley et al. [6] with 2 patients, and Chohan et al. [29] with only 1 patient, suggesting an NF2 rate of 33%, 50%, and 100%, respectively.

Table 3.

PM patients from literature review with concomitant NF2

 PM patients from literature review with concomitant NF2
 PM patients from literature review with concomitant NF2

This study supports the relationship between NF2 and pediatric cerebral meningioma but at a lower concomitant rate from 0 to 13%, taking into consideration our original data and literature review of available data. Hence, the incidence of NF2 in the pediatric population with meningioma is far less and in contrast with some isolated single-center studies, which suggest a higher prevalence rate of 33%, 50%, and 100%, possibly due to the small number of patients in these isolated reports.

This study also reinforces data from previous studies that gross total resection (Simpson grade I), when possible, is associated with a better prognosis and neurologic outcome for PMs, which are not associated with NF2. Radiation therapy is reserved for residual lesions, high-grade/malignant lesions, and PMs, which are associated with NF2, as they turned out to be more aggressive with the worse prognosis.

Radiation-induced meningiomas are often aggressive and likely to be multiple; they also have a high recurrence rate [14]. In our series, no patient had a previous history of radiation therapy for any oncological disease, and this may have contributed to the zero-recurrence rate coupled with the impressive clinical outcome in general.

Limitations of Article

A larger number of patients with longer follow-up periods are required to better understand the clinical features of these patients and obtain a clearer prognosis; also, some articles in the literature review did not state the clinical outcome of symptoms but rather if there were recurrence of the tumor or not, while others did not provide information about the history of NF2.

The authors thank Mrs. Elsie Dei Anane for the editorial support.

Ethical approval for this study was not required by the Ethics Committee of the Department of Pediatric Neurosurgery of Xuanwu Hospital in accordance with national guidelines. Written informed consent was obtained from the parent/legal guardian of the patient for publication of the details of their medical case and any accompanying images.

All the authors declare no form of conflict of interest.

The authors did not receive any funding for research and preparation of data or the manuscript, but if manuscript is approved, payment for publication would be funded by the Department of Pediatric Neurosurgery of Xuanwu Hospital.

Isabella Opoku: collection of data, data analysis, discussion, literature review, and conclusion. Liu Yang and Peng Sun: collection of data and data analysis. Mading Zhou, Yutong Liu, Jian Ren, Jianxin Du, and Ling Feng: data analysis, discussion, and literature review. Gao Zeng: supervised collection of data, data analysis, discussion, literature review, and conclusion.

The data that support the findings of this study are included in this article and available to editors, reviewers, and readers without any restrictions. Further inquiries can be directed to the corresponding author.

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