Shaikh Nawal, Dixit Karan, Raizer Jeffrey
Northwestern University Feinberg School of Medicine, Chicago, USA.
F1000Res. 2018 Apr 24;7. doi: 10.12688/f1000research.13674.1. eCollection 2018.
Meningiomas are the most common adult primary intracranial tumor. Despite their higher incidence, there have not-until recently-been as many advances in understanding and managing meningiomas. Thus far, two broad classes of meningiomas have emerged on the basis of their mutational profile: those driven by neurofibromatosis 2 (NF2) inactivation and those with non-NF2 driver gene alterations, such as mammalian target of rapamycin and Hedgehog, Wingless/b-catenin, Notch, transforming growth factor-b receptor, mitogen-activated protein kinase, and phospholipase C pathway alterations. In addition to improvements in molecular diagnostics, advances in imaging are being studied to better predict tumor behavior, stratify risk, and potentially monitor for disease response. Management consists primarily of surgery and radiation therapy and there has been limited success from medical therapies, although novel targeted agents are now in clinical trials. Advances in imaging and understanding of the genetic makeup of meningiomas demonstrate the huge potential in revolutionizing the classification, diagnosis, management, and prognosis of meningiomas..
脑膜瘤是最常见的成人原发性颅内肿瘤。尽管其发病率较高,但直到最近,在脑膜瘤的认识和治疗方面进展并不多。迄今为止,根据其突变特征已出现两大类脑膜瘤:一类由神经纤维瘤病2型(NF2)失活驱动,另一类具有非NF2驱动基因改变,如雷帕霉素靶蛋白、刺猬信号通路、无翅型/β-连环蛋白、Notch、转化生长因子-β受体、丝裂原活化蛋白激酶和磷脂酶C途径改变。除了分子诊断方面的改进,正在研究成像技术的进展,以更好地预测肿瘤行为、分层风险并潜在地监测疾病反应。治疗主要包括手术和放射治疗,尽管新型靶向药物目前正在进行临床试验,但药物治疗取得的成功有限。成像技术的进步以及对脑膜瘤基因组成的了解表明,在彻底改变脑膜瘤的分类、诊断、治疗和预后方面具有巨大潜力。