血管母细胞瘤和其他血管起源的脑或脊髓瘤

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Updated: Jul 23, 2025

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Ignazio G Vetrano¹, Andrea Gioppo², Giuseppe Faragò²

¹Neurovascular Surgery Unit, Department of Neurosurgery, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.

Advances in experimental medicine and biology

|July 15, 2023

概括

血管母细胞瘤 (HBs) 是一种罕见的,生长缓慢的瘤. 这篇综述涵盖了它们的流行病学,遗传学和治疗,强调对零星病例和与希佩尔-林道氏病有关的病例的诊断和管理.

科学领域:

神经学神经学
在瘤学瘤学.
遗传学是一个遗传学.

背景情况:

血管母细胞瘤 (HBs) 是一种罕见的,良性,高度血管化的瘤 (世卫组织I级),主要影响小脑和脊髓.
虽然HBs往往是零星的 (57-75%),但它是希佩尔-林道氏病 (VHL) 的关键特征,这是由VHL基因突变引起的自体主导性疾病.
VHL疾病使个体易患各种瘤,包括HBs,细胞癌和染细胞瘤.

研究的目的:

提供一个系统的流行病学,临床表现和中枢神经系统HBs. HBs. 组织病理学特征的综述.
详细说明背后的遗传和分子生物学,包括零星和VHL相关的HBs.
概述HBs患者目前的治疗策略和随访管理.

主要方法:

对零星和VHL相关的HBs流行病学数据的审查.
对临床,组织病理学和神经放射学特征的分析.
检查遗传突变 (VHL基因) 和分子生物学.
综合有关诊断方式的信息,主要是磁共振成像 (MRI).
评估治疗选择,包括手术,观察,放射治疗和内血管治疗.

主要成果:

肝炎是成年人中最常见的原发性小脑瘤,在2-10%的脊髓瘤中发生.
散发性HBs通常影响40岁左右的个体,而与VHL相关的HBs通常在较年轻时出现.
增加的内压力和小脑体征是常见的症状;MRI是诊断和随访的黄金标准.
手术是主要的治疗方法,观察和放射治疗在治疗中发挥作用,特别是在VHL患者中.

结论:

对HB流行病学,遗传学和临床行为的全面了解对于有效的管理至关重要.
磁力共振成像对于诊断和监测至关重要,指导治疗决策.
治疗策略必须是个性化的,平衡风险和益处,手术是症状性HBs的主要支柱.

关键词:

血管肉瘤是一种血管肉瘤.在光中光.在HIF中,我们可以看到HIF.血液血管母细胞瘤血管内皮瘤 (hemangioendothelioma) 是一种血管内皮瘤.在ICG中使用ICG.孤独的纤维性瘤 / 血管皮细胞瘤这是VHLVHL.·希普尔-林多病是一种疾病.

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