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

• Published in: Oncogene Vol. 40; no. 5; pp. 875 - 884
• Main Authors: Fountain, Daniel M., Smith, Miriam J., O’Leary, Claire, Pathmanaban, Omar N., Roncaroli, Federico, Bobola, Nicoletta, King, Andrew T., Evans, Dafydd Gareth
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 04.02.2021; Nature Publishing Group
• Subjects: 1-Phosphatidylinositol 3-kinase; 45/23; 631/136/142; 631/67/68; 64/110; Apoptosis; Brain cancer; Brain tumors; Care and treatment; Cell Biology; Chromosome 22; Development and progression; Embryogenesis; Embryology; Genetic aspects; Genetic variation; Genomic instability; Genotype; Health aspects; Hedgehog protein; Hedgehog Proteins - genetics; Human Genetics; Humans; Internal Medicine; KLF4 protein; Kruppel-Like Factor 4; Kruppel-Like Transcription Factors - genetics; Medicine; Medicine & Public Health; Meningeal Neoplasms - genetics; Meningeal Neoplasms - pathology; Meninges; Meningioma; Meningioma - genetics; Meningioma - pathology; Mutation - genetics; Oncology; Pathogenesis; Phenotype; Phenotypes; Phosphatidylinositol 3-Kinases - genetics; Proto-Oncogene Proteins c-akt - genetics; Review; Review Article; Signal Transduction - genetics; United Kingdom
• ISSN: 0950-9232; 1476-5594; 1476-5594
• DOI: 10.1038/s41388-020-01568-6

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.