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[脊髓髓内肿瘤与神经纤维瘤病]

[Intramedullary spinal cord tumors and neurofibromatosis].

作者信息

Kushel' Yu V, Belova Yu D, Tekoev A R

机构信息

Burdenko Neurosurgical Institute, Moscow, Russia.

出版信息

Zh Vopr Neirokhir Im N N Burdenko. 2017;81(1):70-73. doi: 10.17116/neiro201780770-73.

Abstract

UNLABELLED

The article addresses the issue of a combination and relationship between intramedullary spinal cord tumors (IMSCTs) and neurofibromatosis (NF).

AIM

To study, based on large clinical material, features of the prevalence and biological nature of intramedullary spinal cord tumors in neurofibromatosis patients in different age groups.

MATERIAL AND METHODS

We analyzed the data of the largest series of patients from all age groups who underwent surgery for spinal cord intramedullary tumors (541 patients; 586 surgeries; age, 2 months to 72 years).

RESULTS

Our findings support a potential pathogenetic relationship between intramedullary tumors and neurofibromatosis. Astrocytoma was a predominant intramedullary tumor in patients with NF-1, while ependymoma predominated in patients with NF-2. IMSCTs combined with NF-1 occur predominantly in children and adolescents, while a combination with NF-2 is typical of young adults. Our findings confirm the fact that IMSCT surgery is required for a small number of NF patients.

摘要

未标注

本文探讨脊髓髓内肿瘤(IMSCTs)与神经纤维瘤病(NF)的合并情况及关系问题。

目的

基于大量临床资料,研究不同年龄组神经纤维瘤病患者脊髓髓内肿瘤的患病率特征及生物学特性。

材料与方法

我们分析了来自所有年龄组接受脊髓髓内肿瘤手术的最大系列患者的数据(541例患者;586次手术;年龄2个月至72岁)。

结果

我们的研究结果支持髓内肿瘤与神经纤维瘤病之间存在潜在的发病机制关系。星形细胞瘤是NF-1患者中主要的髓内肿瘤,而室管膜瘤在NF-2患者中占主导。IMSCTs与NF-1合并主要发生在儿童和青少年,而与NF-2合并则在年轻人中较为典型。我们的研究结果证实了少数NF患者需要进行IMSCT手术这一事实。

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