Meningiomas of the central nervous system occurring below the age of 17: report of 24 cases not associated with neurofibromatosis and review of literature

The objective of this work was to gain more insight into the controversial characteristics of meningiomas occurring during childhood and adolescence. Management of meningiomas is an important field in pediatric neurosurgery. Every pediatric neurosurgeon has tried to resolve the problems relating to the clinical characteristics, biological behavior and outcome of this interesting and almost benign pathology, which rarely occurs in the first two decades of life. The records on central nervous system (CNS) tumors held by the two major neurosurgery centers of Tehran Medical University and Arad General Hospital were prospectively collected during last 15 years. Complete medical records are available for all 24 cases, and long-term follow-up was achieved 19 patients. All the cases were diagnosed and treated after the introduction of computed tomographic (CT) scanning. Angiography and magnetic resonance imaging (MRI) were performed as complementary studies in some cases. The sample consisted of 13 girls and 11 boys. The age range at the time of diagnosis varied between 2 and 17 years, with a mean of 9.47 and standard deviation of 3.43. Fifteen patients were below 10 years of age (62.5%), and 9 of them were between 10 and 17 years old (37.5%). The most common presenting symptoms, in declining order of frequency, were headache, epilepsy and focal neurological deficits. Similar cases associated with neurofibromatosis either at the time of presentation with meningioma or during the follow-up period were excluded (5 cases). The size of the presenting tumor was more than 5 cm in diameter in 17 cases. The locations of the lesions, taken as the site of the presumed widest dural base in each case were: spinal, orbital, ethmoidal and sphenoethmoidal in 1 case each, petroclival in 2, and tentorial or supratentorial in 18 patients. The only predisposing factor in this series of childhood meningiomas was whole-axis irradiation for previous malignancy in the case presenting with cervical intradural meningioma. There have been no surgical deaths, and gross total excision of the lesions was achieved in 21 cases. Tumor recurrence was observed during the follow-up period in 6 cases (25%). The follow-up period varied between 2 and 165 months, with a median interval of 130.2 months. This series of pediatric CNS meningiomas comprises almost 1.08% of all meningiomas operated on by the authors during the last 15 years and it also accounts for about 1.1% of all pediatric CNS tumors encountered. This series of patients has certain characteristics regarding sex distribution, unusual size, peculiar localizations, special histological features and benign clinical behavior distinguishing it from other series reported in the literature. Received: 15 February 1999 Revised: 18 July 1999