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颅内胶质母细胞瘤有症状性脊柱转移:与 GFAP 表达相关的临床特征和发病机制。

Symptomatic spinal metastases of intracranial glioblastoma: clinical characteristics and pathomechanism relating to GFAP expression.

机构信息

Department of Neurosurgery, University Hospitals Schleswig-Holstein, Campus Kiel, Arnold-Heller-Straße 3, 24105 Kiel, Germany.

出版信息

J Neurooncol. 2011 Jan;101(2):329-33. doi: 10.1007/s11060-010-0257-y. Epub 2010 Jun 13.

DOI:10.1007/s11060-010-0257-y
PMID:20549302
Abstract

To demonstrate clinical characteristics of symptomatic spinal metastases of intracranial glioblastoma multiforme (GBM) and different spreading mechanisms relating to astrocytic cell differentiation, we present an extraordinary case of a 47-year-old patient with rapid progressive paraplegia due to coincident intramedullary and leptomeningeal dissemination of a supratentorial GBM. Serial biopsies of the intracranial, leptomeningeal, and intramedullary GBM lesions of our patient were analyzed for glial fibrillary acidic protein (GFAP). Furthermore, we present 19 additional cases of intracranial GBM with symptomatic spinal seeding, identified through literature review. GFAP expression was high in intracranial and intramedullary tumors, but low in leptomeningeal dissemination of our patient. Mean patient age was 45 years. Mean interval between identification of spinal metastases and death was 4.5 months. Mean overall survival was 18.6 months. Location of symptomatic spinal metastases was more frequently leptomeningeal (14 cases) than intramedullary (7 cases). The case presented herein supports the hypothesis of higher incidence of low GFAP expression in GBM cells in leptomeningeal manifestations after primary intracranial GBM. Because of the proposed tendency for early leptomeningeal spread from primary tumors with low astrocytic differentiation (low GFAP expression), patients with these tumors should be followed more closely to identify leptomeningeal tumor progression early on. Early identification of leptomeningeal spread could enable these patients to benefit from radiation therapy before they develop severe neurological deficits, which might translate into longer acceptable quality of life for these mostly young patients. This is an important finding, but further prospective studies are needed to verify our observations.

摘要

为了展示颅内胶质母细胞瘤(GBM)的症状性脊髓转移的临床特征和与星形胶质细胞分化相关的不同扩散机制,我们报告了一例 47 岁患者的特殊病例,该患者因幕上 GBM 的脊髓内和软脑膜同时播散而迅速进展为截瘫。对我们患者的颅内、软脑膜和脊髓内 GBM 病变的连续活检进行了神经胶质纤维酸性蛋白(GFAP)分析。此外,我们通过文献回顾,报告了 19 例伴有症状性脊髓播散的颅内 GBM 病例。GFAP 在颅内和脊髓内肿瘤中的表达较高,但在我们患者的软脑膜播散中表达较低。患者的平均年龄为 45 岁。从发现脊髓转移到死亡的平均间隔时间为 4.5 个月。总生存期平均为 18.6 个月。症状性脊髓转移的位置更常见于软脑膜(14 例)而不是脊髓内(7 例)。本文报告的病例支持这样一种假设,即在原发性颅内 GBM 后,GBM 细胞在软脑膜表现中 GFAP 表达较低的发生率更高。由于原发性肿瘤中具有低星形胶质分化(低 GFAP 表达)的肿瘤更倾向于早期软脑膜扩散,因此应更密切地随访这些肿瘤患者,以便及早发现软脑膜肿瘤进展。早期发现软脑膜扩散可以使这些患者在出现严重神经功能缺损之前受益于放射治疗,这可能会使这些主要为年轻患者的生活质量得到更长时间的接受。这是一个重要的发现,但需要进一步的前瞻性研究来验证我们的观察结果。

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