Purpose of Review

Meningioma is a common intracranial neoplasm currently classified in 15 histologic subtypes across 3 grades of malignancy. First-choice therapy for meningioma is maximum safe resection for grade I tumors, and surgery plus optional and mandatory adjuvant radiotherapy for grade II and III, respectively, given the increased rate of recurrence even in the event of complete resection. The WHO 2016 histopathologic grading of meningioma has been questioned due to subjectivity and its controversial predictive power for recurrence.

Recent Findings

Novel DNA methylation profiling has simplified classification into six classes that seem to improve prognostic accuracy.

Summary

We review five main topics of molecular biology research regarding tumorigenesis and natural history of meningioma from the clinician’s perspective: the histopathologic diagnostic features and pitfalls of the current tumor classification; the molecular integrated diagnosis supported by identification of genetic alterations and DNA methylation profiling; the general landscape of the various signaling pathways involved in meningioma formation; the pathogenic theories of the peri-tumoral edema present in meningioma and its therapy implications; and a summarized review on the current treatments and plausible targeted therapies directed to meningioma. It seems likely that molecular assessment will be introduced within the next update of the WHO classification of meningiomas, acknowledging the promising value of DNA methylation profiling. This integrated diagnostic protocol will simplify tumor subtype categorization and provide improved accuracy in predicting recurrence and outcome. Although much effort is being done in identifying key gene mutations, and elucidating specific intracellular signaling pathways involved in meningioma tumorigenesis, effective targeted therapies for recurrent meningiomas are still lacking.

中文翻译：

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脑膜瘤分子生物学实用概述

审查目的

脑膜瘤是一种常见的颅内肿瘤，目前分为3个级别的恶性肿瘤，分为15种组织学亚型。脑膜瘤的首选治疗是最大的安全切除I类肿瘤的方法，并且手术以及对II和III级分别进行选择性和强制性辅助放疗的患者，即使完全切除也可以提高复发率。世卫组织2016年脑膜瘤组织病理学分级因主观性及其对复发的预测能力而受到质疑。

最近的发现

新颖的DNA甲基化分析已将分类简化为六类，似乎可以提高预后准确性。

概要

我们从临床医生的角度回顾了有关脑膜瘤的发生发展和自然史的分子生物学研究的五个主要主题：当前肿瘤分类的组织病理学诊断特征和缺陷；分子综合诊断得到遗传变异和DNA甲基化图谱的鉴定支持；与脑膜瘤形成有关的各种信号通路的总体情况；脑膜瘤中肿瘤周围水肿的病原学理论及其治疗意义；以及针对脑膜瘤的当前治疗方法和合理的靶向治疗方法的综述。世卫组织对脑膜瘤的分类的下一次更新似乎将引入分子评估，这肯定了DNA甲基化谱图的前景。该综合诊断方案将简化肿瘤亚型的分类，并提高预测复发和预后的准确性。尽管在确定关键基因突变和阐明与脑膜瘤肿瘤发生有关的特定细胞内信号传导途径方面已付出了很大的努力，但仍缺乏有效的针对复发性脑膜瘤的靶向治疗方法。