Meningiomas are the most prevalent primary intracranial tumors. The majority are benign but can undergo dedifferentiation into advanced grades classified by World Health Organization (WHO) into Grades 1 to 3. Meningiomas’ tremendous variability in tumor behavior and slow growth rates complicate their diagnosis and treatment. A deeper comprehension of the molecular pathways and cellular microenvironment factors implicated in meningioma survival and pathology is needed. This review summarizes the known genetic and epigenetic aberrations involved in meningiomas, with a focus on neurofibromatosis type 2 (NF2) and non-NF2mutations. Novel potential biomarkers for meningioma diagnosis and prognosis are also discussed, including epigenetic-, RNA-, metabolomics-, and protein-based markers. Finally, the landscape of available meningioma-specific animal models is overviewed. Use of these animal models can enable planning of adjuvant treatment, potentially assisting in pre-operative and post-operative decision making. Discovery of novel biomarkers will allow, in combination with WHO grading, more precise meningioma grading, including meningioma identification, subtype determination, and prediction of metastasis, recurrence, and response to therapy. Moreover, these biomarkers may be exploited in the development of personalized targeted therapies that can distinguish between the 15 diverse meningioma subtypes.
脑膜瘤是最常见的原发性颅内肿瘤。多数为良性,但可发生去分化而进展为高级别,世界卫生组织(WHO)将其分为1至3级。脑膜瘤在肿瘤行为上的巨大差异及缓慢的生长速度使其诊断和治疗复杂化。需要更深入地理解与脑膜瘤生存和病理相关的分子通路及细胞微环境因素。本综述总结了已知的与脑膜瘤相关的遗传和表观遗传异常,重点关注2型神经纤维瘤病(NF2)和非NF2突变。同时讨论了用于脑膜瘤诊断和预后的新型潜在生物标志物,包括基于表观遗传学、RNA、代谢组学和蛋白质的标志物。最后,概述了现有的脑膜瘤特异性动物模型。利用这些动物模型有助于规划辅助治疗,可能为术前和术后决策提供支持。新型生物标志物的发现,结合WHO分级,将实现更精确的脑膜瘤分级,包括脑膜瘤的识别、亚型确定以及转移、复发和治疗反应的预测。此外,这些生物标志物可用于开发能够区分15种不同脑膜瘤亚型的个性化靶向治疗。