Pediatric meningiomas: A literature review and diagnostic update

BACKGROUND: Meningiomas have always represented the most frequently observed primary central nervous system (CNS) tumor in adults. Multiple advances concerning the genetic and epigenetic characterizations of adult meningiomas have been made over the last few years, and a new proposition for integrated histo-molecular grading has recently been offered in the literature. Pediatric meningiomas represent a very small proportion of all diagnosed meningiomas. New literature has determined that pediatric meningiomas are clinically, histopathologically, genetically, and epigenetically distinct from their adult counterparts. Herein, we reviewed and performed a synthesis of literature investigating pediatric meningiomas. We then compared and contrasted pediatric meningiomas with their adult counterparts. METHODS: We performed an extensive review of cases from English-language literature available in Pubmed using the keywords “pediatric” and “meningioma” as well as “children” and “meningioma”. We reviewed and analyzed fifty-six papers that include 498 cases. RESULTS: This literature review revealed that pediatric meningiomas differ from their adult counterparts clinically (location, sex ratio) and also in terms of etiology (germline mutations), histopathology (a greater incidence of clear cell subtype), molecular biology, and epigenetics. CONCLUSIONS: Pediatric meningiomas are, like other brain tumors (such as low-grade and high-grade gliomas), clinically and biologically different from their adult counterparts. Further studies are needed to better understand the tumorigenesis of pediatric meningiomas and to optimize their stratification in terms of outcome and therapeutic strategy.