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小儿脑膜瘤:发病机制与治疗的最新认识。

Pediatric Meningiomas: Current Insights on Pathogenesis and Management.

机构信息

Department of Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Catholic University, Rome, Italy.

Department of Neurosurgery, Military Hospital, Hamburg, Germany.

出版信息

Acta Neurochir Suppl. 2023;135:69-74. doi: 10.1007/978-3-031-36084-8_12.

Abstract

Meningiomas are rare tumors in children, ranging from 0.4 to 4% of intracranial tumors. Differently from their adult counterpart, pediatric meningiomas (PMs) often show peculiar aspects such as the development of tumoral cysts, the involvement of the intraventricular space, and missing attachment to the dura mater. The most important difference with adults is represented by the high incidence of WHO grade II and III variants, which can account for more than 70% of cases. The prognosis of PMs mainly depends on the initial surgical resection because radiotherapy, which is the main treatment option in the case of tumor recurrence or progression, does not seem to increase the relapse free survival and the overall survival, and chemotherapy still misses specific and effective protocols.On these grounds, the need to better understand these tumors, to favor an appropriate multidisciplinary management, is particularly felt. The present review is focused on the advances on the pathogenesis, the molecular aspects, and the managements of PMs, with the goal to improve the knowledge of these challenging neoplasms.

摘要

脑膜瘤在儿童中较为罕见,占颅内肿瘤的 0.4%至 4%。与成人脑膜瘤不同,儿童脑膜瘤(PMs)常表现出一些特殊的特征,如肿瘤性囊肿的形成、脑室空间的受累以及与硬脑膜的缺失附着。与成年人最重要的区别在于,WHO 分级 II 和 III 变体的发生率较高,超过 70%的病例属于这一级别。PMs 的预后主要取决于初始手术切除,因为放疗是肿瘤复发或进展时的主要治疗选择,但似乎并不能提高无复发生存率和总生存率,而化疗仍缺乏特异性和有效的方案。基于这些原因,特别需要更好地了解这些肿瘤,以支持适当的多学科管理。本综述重点介绍了 PMs 的发病机制、分子方面和治疗管理方面的进展,旨在提高对这些具有挑战性的肿瘤的认识。

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