Article
Prognostic variables in childhood meningioma: summary of 21 cases with attention to associated neurofibromatosis 2 and literature overview
Search Medline for
Authors
-
Friederike Fritzsche
- Klinik und Poliklinik für Neurochirurgie, Universitätsklinikum Hamburg-Eppendorf
-
Gertrude Kammler
- Klinik und Poliklinik für Neurochirurgie, Universitätsklinikum Hamburg-Eppendorf
-
Manfred Westphal
- Klinik und Poliklinik für Neurochirurgie, Universitätsklinikum Hamburg-Eppendorf
-
Pedram Emami
- Klinik und Poliklinik für Neurochirurgie, Universitätsklinikum Hamburg-Eppendorf
| Published: | May 13, 2014 |
|---|
Outline
Text
Objective: Meningiomas in children and adolescents are a rare condition and still poorly investigated clinical entity. They occur spontaneously or are associated with special risk factors like radiation therapy, neurofibromatosis 1 and 2 or other genetic predisposition. The purpose of this series was to determine prognostic variables and to point out the association with neurofibromatosis 2 (NF2).
Method: Clinical data of 21 patients with age from 0 to 17 yrs was analysed retrospectively. The patients underwent surgery for central nervous system meningioma at our clinical centre during a period of 1981-2010. Data such as age, sex, tumour location, presence of NF2, histopathology and recurrence was collected. A review of the literature in pubmed, cochrane and medline was base for additional specific discussion.
Results: The study group included 9 females and 12 males, i.e. 1:1.33. Mean age was 10,7 yrs at surgery. 5 children had evidence of NF2 (23,8%). Tumour localization showed broad variety (orbita, skull base, ventricle, convexity, spinal cord). The histopathology revealed 17 WHO grade I meningiomas (80,9%) and 4 WHO grade II meningiomas (19,1%). 3 patients with WHO grade I meningioma showed association with NF2 (2 meningothelial, 1 transitional). In the group of grade II meningiomas, 2 cases were associated with NF2 (50,0%), both showing atypical subtype. 5 tumours recurred (23,8%), none was associated with NF2.
Conclusions: To establish an epidemiological profile, firstly shown is a male predominance in this series. Recurrences in pediatric meningiomas were not associated with NF2 whereas the current literature classifies these patients as special risk category needing close follow-up. Patients with NF2 seemed to have a risk to develop WHO grade II meningiomas. After additional review of the literature, surgery seems to be the first line treatment regardless of tumour localization. The extent of tumour resection is an essential prognostic factor in meningiomas in the paediatric age group.
