BACKGROUND Meningiomas are usually considered benign lesions, however a part of them can show very aggressive behavior with tendency to metastasize. This subgroup is known as high-grade meningiomas (HGM). Due to the rarity of the disease, effective medical treatments are lacking, especially at the time of recurrence. We aim to describe the clinical, radiological and molecular features of a large population of HGM diagnosed between 2010 and 2018. The secondary aim was to evaluate survival (PFS) and overall survival (OS). MATERIAL AND METHODS We collected clinical and survival data from primitive WHO grade II and III meningioma patients treated at Fondazione IRCCS Istituto Neurologico Carlo Besta from January 2010 to December 2018. Records were collected on a web-based platform (Microsoft Excel) that was customized for this study. The database, contained epidemiological, diagnostic (radiological and histological/molecular), surgical, therapeutic and recurrence information, as well as survival data. RESULTS 183 patients (105 females and 78 males), with median age of 58 years (25–88), were included; 168 were atypical, 12 anaplastic, 3 rhabdoid. Overall, m-PFS was 4.2 years, and m-OS was 10.3 years. Gross-total resection had a 5-year survival rate of 95% compared with subtotal/partial resection (86% and 67%) (p=0.002). Higher expression of Ki-67/MIB-1 seems associated with higher risk of death (HR:1.06 with 95% CI, 1.00–1.12, p=0.03). No statistically significant differences were seen in survival between the group managed with a wait-and-see strategy vs the group treated with RT/SRS while a difference on PFS was seen (4.1 years vs 5.2 years p=0.03). After second recurrence, the most employed treatments were systemic therapies with a very limited effect on disease control. CONCLUSION Data confirmed the aggressive behavior of HGM. The extent of resection seems to correlate with a favorable outcome regardless histological subtypes. The role of RT/SRS remains controversial, with no statistically significant impact on OS but a possible role on PFS. At relapse, no chemotherapies are able to achieve disease control and recurrent HGM remains the real challenge for future research, focusing on biological/molecular predictors in order to achieve a patient-tailored treatment.This retrospective study allowed to analyzed the largest national recent series of aggressive meningiomas with the purpose of identify relevant outcome measures for future prospective studies. Our findings could be important to give a snapshot of the current attitude to treat aggressive meningiomas but also to inspire national and international collaboration in order to provide evidence-based management strategies trying to obtain a standard of care.

中文翻译：

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P14.57 WHO II 级和 III 级脑膜瘤：大型单机构回顾性系列

背景技术脑膜瘤通常被认为是良性病变，但是它们中的一部分可以表现出非常具有侵袭性的行为并具有转移的倾向。该亚组被称为高级别脑膜瘤 (HGM)。由于这种疾病的罕见性，缺乏有效的药物治疗，尤其是在复发时。我们旨在描述 2010 年至 2018 年间诊断出的大量 HGM 人群的临床、放射学和分子特征。次要目的是评估生存期 (PFS) 和总生存期 (OS)。材料和方法 我们收集了 2010 年 1 月至 2018 年 12 月在 Fondazione IRCCS Istituto Neurologico Carlo Besta 接受治疗的原始 WHO II 级和 III 级脑膜瘤患者的临床和生存数据。记录是在一个基于 Web 的平台 (Microsoft Excel) 上收集的，该平台为这项研究。数据库，包含流行病学、诊断（放射学和组织学/分子）、手术、治疗和复发信息，以及生存数据。结果共纳入183例患者（105例女性，78例男性），中位年龄58岁（25-88岁）；168 不典型，12 间变性，3 横纹肌。总体而言，m-PFS 为 4.2 年，m-OS 为 10.3 年。与次全/部分切除（86% 和 67%）相比，大全切除的 5 年生存率为 95%（p=0.002）。Ki-67/MIB-1 的较高表达似乎与较高的死亡风险相关（HR：1.06，95% CI，1.00-1.12，p=0.03）。采用观望策略管理的组与接受 RT/SRS 治疗的组之间的生存率没有统计学显着差异，而 PFS 存在差异（4.1 年对 5.2 年 p=0.03）。第二次复发后，最常用的治疗是全身治疗，对疾病控制的影响非常有限。结论 数据证实了 HGM 的攻击性行为。无论组织学亚型如何，切除范围似乎都与良好的结果相关。RT/SRS 的作用仍然存在争议，对 OS 没有统计学意义的影响，但可能对 PFS 有影响。在复发时，没有任何化学疗法能够实现疾病控制，并且复发性 HGM 仍然是未来研究的真正挑战，重点是生物/分子预测因子，以实现针对患者的治疗。这项回顾性研究允许分析最大的国家近期系列侵袭性脑膜瘤，目的是为未来的前瞻性研究确定相关的结果指标。