The spatial phenotype of genotypically distinct meningiomas demonstrate potential implications of the embryology of the meninges

Abstract

Meningiomas are the most common primary brain tumor and their incidence and prevalence is increasing. This review summarizes current evidence regarding the embryogenesis of the human meninges in the context of meningioma pathogenesis and anatomical distribution. Though not mutually exclusive, chromosomal instability and pathogenic variants affecting the long arm of chromosome 22 (22q) result in meningiomas in neural-crest cell-derived meninges, while variants affecting Hedgehog signaling, PI3K signaling, TRAF7, KLF4, and POLR2A result in meningiomas in the mesodermal-derived meninges of the midline and paramedian anterior, central, and ventral posterior skull base. Current evidence regarding the common pathways for genetic pathogenesis and the anatomical distribution of meningiomas is presented alongside existing understanding of the embryological origins for the meninges prior to proposing next steps for this work.

Fig. 1. The spatial phenotype of genotypically distinct meningioma and embryology of the meninges.

A Anatomical depiction of meninges with brain and spinal cord removed displaying skull base, sagittal, and convexity regions including tentorium cerebelli on the right side. B distribution of meningioma by pathogenic variant gene pathway. C meningeal embryonic development by the tissue of origin.