Multiple faces of children and juvenile meningiomas: A report of single-center experience and review of literature

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Abstract

Objective

Pediatric meningiomas are extremely rare tumors often associated with various medical conditions. This report is an attempt to analyze the clinical behavior, pathological presentation and recommended management of both isolated and neurofibromatosis type 2 (NF2) related pediatric meningiomas in comparison to their adult counterparts.

Methods

The investigated group consisted of three male and six female patients suffering from meningiomas of the central nervous system, who were hospitalized at our department in years 2001–2012.

Results

In our group three children were diagnosed with NF2. Two were released with a suspicion of that disease. Three patients had multiple meningiomas at the first presentation. The most common manifestation were focal neurological deficits (47%). Meningiomas were mostly located at the brain convexity (29%). Histological examination revealed the domination of fibrous type in our group (29%). Apart from surgical management a preoperative embolization of the tumors' vessels was implemented in two children. Another three children underwent postoperative radiotherapy (RTh), one received a chemotherapy (ChTh).

Conclusion

Meningiomas in children differ from their counterparts in adults. Due to common coexistence of pediatric meningiomas and neurofibromatosis type 2, this group of patients should be taken under strict control, because of high risk of tumor recurrence.

Introduction

Although meningiomas are uncommon in children and adolescents they are undoubtedly an important and challenging tumors for pediatric neurosurgeons. Not only because they differ from their adult counterparts, but also because they are prone to accompany several medical conditions: neurofibromatosis type 2 (NF2), Rubenstein–Taybi syndrome and Castleman disease [1]. Meningiomas amount to 0.4–4.6% of all primary central nervous tumors (CSN) in pediatric group [2]. These may explain why their biological behavior and therapeutical guidelines still remain uncertain.

This report is an attempt to analyze the clinical behavior, pathological picture and recommended management of pediatric meningiomas in comparison to their adult counterparts and to elucidate the differences between sporadic and NF2-related meningiomas on the basis of own experience and literature.

Section snippets

Material and methods

Over a 11-year period (2001–2012) nine patients younger than 18 years were hospitalized at the Department of Neurosurgery at University Children's Hospital, Jagiellonian University Medical College. Altogether there were twenty-four meningiomas in the investigated group. All patients were analyzed retrospectively. The data collected from the hospital records included age, sex, clinical presentation, extent of resection, number of recurrences, associated factors (NF2) and histopathology.

Clinical data

Nine children with meningiomas underwent a surgery in our department in years 2001–2012. We noticed a strong female predominance with six girls and three boys in the study group. Mean age at the first admission to the department was 11.3 years. The youngest child was 5 years old. The mean length of symptoms was 9.5 month ranging from 3 weeks to 3 years. Focal neurological deficits were the most common manifestation of a meningioma in our group (47%), followed by the symptoms of raised

Pediatric vs. adult meningiomas

Pediatric meningiomas in comparison to meningiomas in adults show differences both in clinical and biological aspects. These tumors making up 30% of all primary central nervous system neoplasms in adults, account for only 0.4–4.6% of pediatric ones [2]. According to Central Brain Tumor Registry of the United States, the number of meningiomas raises with age [3]. This data is in line with our results revealing one vs. five children with meningioma in age groups 0–6 and 13–18 years old

Conclusion

Meningiomas in children create a heterogenous group and differ meaningfully from their adult counterparts. Because of that, an individual therapeutic approach is necessary. In pediatric group due to strong association of meningiomas and neurofibromatosis type 2, each child should be investigated thoroughly so that not to overlook silently growing vestibular schwannoma. Resection is the procedure of choice in symptomatic pediatric meningiomas and should be attempted both in sporadic and in

Funding

None declared.

Conflicts of interest

None declared.

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