Central nervous system meningiomas in the first two decades of life: a clinicopathological analysis of 87 patients

Abstract

Object: The occurrence of meningiomas in children younger than 20 years of age is rare, accounting for less than 3% of all childhood tumors of the central nervous system. The authors of this study sought to add to the limited available information regarding clinicopathological factors that influence outcome, disease progression, and survival in children with meningiomas.

Methods: Eighty-seven cases of childhood meningiomas were identified and classified according to World Health Organization (WHO) 2000 criteria. In addition to the WHO classification, the following potential prognostic factors were analyzed: age, sex, extent of resection, history of radiotherapy, diagnosis of neurofibromatosis Type 2 (NF2) or other inherited syndromes, and the presence of a comorbidity. There was a sex predilection for male patients (35 females and 52 males). Patient age ranged from 5 months to 20 years (mean 14 years). The most common clinical presentations were seizures (33%), headaches (13%), ataxia (10%), and hemiparesis (10%). Nine patients had NF2 and two had Gorlin syndrome. Seven patients had undergone radiotherapy for a prior neoplasm. Tumor location was supratentorial in 64% of the patients, infratentorial in 16%, intraventricular in 12%, and spinal in 8%. Fifty-three patients (62%) underwent gross-total resection and 28 (33%) underwent subtotal resection. Histopathological analysis revealed 62 (71%) WHO Grade I, 21 (24%) Grade II, and four (5%) Grade III meningiomas. One patient received adjuvant chemotherapy and four received radiotherapy.

Conclusions: Using survival data from this unique patient cohort, the authors found that recurrence-free survival time was significantly related to WHO grade (p = 0.002), but overall survival time was not significantly linked to any of the potential prognostic factors considered in this study (p = 0.06).