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肿瘤性血管内皮细胞增生症:一种具有多灶性表现的临床病理实体。病例报告。

Neoplastic angioendotheliosis: a clinicopathological entity with multifocal presentation. Case report.

作者信息

Ansbacher L, Low N, Beck D, Boarini D, Jacoby C, Cancilla P A

出版信息

J Neurosurg. 1981 Mar;54(3):412-5. doi: 10.3171/jns.1981.54.3.0412.

Abstract

Neoplastic angioendotheliosis is a rare disorder characterized by intravascular neoplastic proliferation of endothelial cells within vessels of all caliber in the meninges and neuropil. Ischemic infarcts of brain and spinal cord result from occlusion of the lumina by neoplastic cells of fibrin thrombi. Transition from reactive to neoplastic endothelium can be identified in many vessels. Steroid therapy can be beneficial in reduction of severity of symptoms, but cannot alter the course of disease. Therapeutic intervention must be undertaken promptly after the diagnosis is confirmed by meningeal and cortical biopsy if the inexorable course of the disease is to be altered.

摘要

肿瘤性血管内皮增生症是一种罕见的疾病,其特征为脑膜和神经纤维网内各种管径血管的内皮细胞发生血管内肿瘤性增殖。脑和脊髓的缺血性梗死是由肿瘤细胞或纤维蛋白血栓阻塞管腔所致。在许多血管中可识别出从反应性内皮向肿瘤性内皮的转变。类固醇治疗有助于减轻症状的严重程度,但无法改变疾病进程。如果要改变这种疾病不可避免的病程,在通过脑膜和皮质活检确诊后必须立即进行治疗干预。

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