Hyperostotic meningiomas in children. A case-based update.

Abstract

Background: Meningiomas are rare tumors in children compared with adults. Their main peculiarities are the frequent convexity or intraventricular location, the common association with neurofibromatosis-2 (NF-2) and the relatively high rate of aggressive and/or hyperostotic variants. Hyperostosis may complicate the surgical management. The goal of this paper is to provide an update on the main characteristics and the management of hyperostotic meningiomas.

Case description: A 7-year-old girl was admitted to our department because of a long-lasting history of left frontal skull bulging. Neuroimaging examinations revealed a huge hyperostotic meningioma with bony invasion and infiltration of the superior sagittal sinus. Genetic tests were positive for NF-2. The tumor (atypical meningioma) was gross totally resected. During the same operation, a custom-made cranioplasty was realized with the help of a frame-based craniectomy. The patient is asymptomatic and under oncological follow-up.

Discussion and conclusion: Although generally rare, hyperostotic meningiomas are relatively common in children and clinically demanding because of their large size, the possible aggressive behavior and the need of a cranioplasty as additional surgical step. Therefore, a careful preoperative surgical planning is required, taking into account that the extent of surgical resection is an important prognostic factor. The long-term outcome is good.