Fountain Daniel M, Young Adam M H, Santarius Thomas
Department of Neurosurgery, Addenbrooke's Hospital, Cambridge, United Kingdom.
Department of Neurosurgery, Addenbrooke's Hospital, Cambridge, United Kingdom.
Handb Clin Neurol. 2020;170:245-250. doi: 10.1016/B978-0-12-822198-3.00044-6.
Malignant meningiomas are WHO Grade III meningiomas representing 1% of all meningiomas. They are comprised of three histologic types: anaplastic, rhabdoid, and papillary. They can arise de novo or as a result of biologic progression of meningiomas of lower histologic grades. The overall survival of patients with WHO grade III meningiomas is 2-3 years. Surgery is the main treatment, while radiotherapy is thought to slow tumor growth. Multiple trials have been attempted on chemotherapeutic agents, hormonal therapies, small molecule and anti-angiogenic agents without robust evidence of efficacy. The rarity of these tumors is the main reason for our patchy understanding of the natural history and lack of effective treatment options. There is an urgent need to develop alternative therapies given the significantly increased risk of complication and co-mordibity associated with repeated surgeries in this population.
恶性脑膜瘤是世界卫生组织(WHO)III级脑膜瘤,占所有脑膜瘤的1%。它们由三种组织学类型组成:间变性、横纹肌样和乳头状。它们可原发出现,也可由组织学分级较低的脑膜瘤生物学进展所致。WHO III级脑膜瘤患者的总生存期为2至3年。手术是主要治疗方法,而放疗被认为可减缓肿瘤生长。针对化疗药物、激素疗法、小分子药物和抗血管生成药物已进行了多项试验,但均未获得有力的疗效证据。这些肿瘤的罕见性是我们对其自然史了解不全面以及缺乏有效治疗选择的主要原因。鉴于该人群重复手术相关并发症和合并症风险显著增加,迫切需要开发替代疗法。