Clear cell meningioma in a child: Case report and systematic literature review

Abstract

Background: Clear Cell Meningioma (CCM) consists in a rare variant of meningiomas that generally occur in children. This uncommon tumor behaves aggressively and recurrence rates are high. Clinical manifestations are atypical, and the treatment generally involves surgery. Methods and Goals: We performed a systematic review in order to define the most common characteristics of this rare tumor. Pubmed and Lilacs were the databases used for the literature search. We included 17 studies that met our criteria. To further our research, we included the case report of a 3-year-old female child with CCM operated in our service. Results: CCMs were more frequent in the pediatric population than in adults, especially in females. Gross tumor resection and MIB-1 index < 3 were the parameters related to a smaller recurrence rate. Subtotal tumor resection was followed by radiotherapy in most adult cases. SMARCE-1 mutation could be found in several patients. Conclusion: CCMs are rare meningiomas that occur especially in children. The aggressiveness and high recurrence rates difficult the management of this disease. There is still a lot to be discussed about these rare tumors, demanding further research.