Rowbottom Hojka, Šmigoc Tomaž, Ravnik Janez
Department of Neurosurgery, University Medical Centre Maribor, 2000 Maribor, Slovenia.
Diagnostics (Basel). 2025 Feb 23;15(5):538. doi: 10.3390/diagnostics15050538.
Meningiomas account for approximately 40% of all primary brain tumors, of which 1.5% are classified as grade 3. Whilst meningiomas are discovered on imaging with high-grade meningiomas being associated with certain imaging features, the final diagnosis is based on histopathology in combination with molecular markers. According to the latest World Health Organization (WHO) Classification of Tumors of the Central Nervous System (CNS), grade 3 should be assigned based on criteria for anaplastic meningiomas, which comprise malignant cytomorphology (anaplasia) that resembles carcinoma, high-grade sarcoma or melanoma; elevated mitotic activity; a TERT promoter mutation and/or a homozygous CDKN2A and/or CDKN2B deletion. Surgery remains the mainstay treatment modality for grade 3 meningiomas, followed by radiotherapy. Limited data are available on the effect of stereotactic radiosurgery and systemic therapy for grade 3 meningiomas; however, studies are underway. Despite optimal treatment, the estimated recurrence rate ranges between 50% and 95% with a 5-year survival rate of 66% and a 10-year estimated survival rate of 14% to 24%.
脑膜瘤约占所有原发性脑肿瘤的40%，其中1.5%被归类为3级。虽然脑膜瘤可通过影像学发现，高级别脑膜瘤具有某些影像学特征，但最终诊断基于组织病理学结合分子标志物。根据世界卫生组织（WHO）最新的中枢神经系统（CNS）肿瘤分类，3级应根据间变性脑膜瘤的标准来判定，这些标准包括类似癌、高级别肉瘤或黑色素瘤的恶性细胞形态（间变）；有丝分裂活性升高；TERT启动子突变和/或纯合性CDKN2A和/或CDKN2B缺失。手术仍然是3级脑膜瘤的主要治疗方式，其次是放疗。关于立体定向放射外科和全身治疗对3级脑膜瘤疗效的数据有限；然而，相关研究正在进行中。尽管进行了最佳治疗，但估计复发率在50%至95%之间，5年生存率为66%，10年估计生存率为14%至24%。
