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原发性中枢神经系统血管肉瘤。

Primary Central Nervous System Angiosarcoma.

机构信息

Department of Neurosurgery, First Hospital of China Medical University, Shenyang, China.

Department of Neurosurgery, First Hospital of China Medical University, Shenyang, China.

出版信息

World Neurosurg. 2019 Dec;132:41-46. doi: 10.1016/j.wneu.2019.08.128. Epub 2019 Aug 27.

Abstract

BACKGROUND

Primary angiosarcoma of the brain is an extremely rare malignant tumor that that arises from vascular endothelial cells of the brain or meninges. The presentation, characteristics, and prognosis of this disease are not well understood. Here we report such a case.

CASE DESCRIPTION

A 68-year-old Chinese man presented with confusion and progressive impairment of right limb movement. Magnetic resonance imaging (MRI) revealed a hemorrhagic lesion in the left frontal lobe. Exploratory surgery revealed a hematoma-like lesion and extensive superficial hemosiderin deposition. The postoperative pathological diagnosis was angiosarcoma. The tumor recurred at 1 month after the operation, with extensive tumor regrowth in the left hemisphere. The patient died after 4 weeks.

CONCLUSIONS

Although accurate preoperative diagnosis of angiosarcoma of the brain is difficult, radiologists and neurosurgeons need to be aware of this rare entity. The prognosis of intracranial angiosarcoma is heterogenous. Intraoperatively, the presence of extensive superficial hemosiderosis may aid in its diagnosis.

摘要

背景

原发性脑血管肉瘤是一种极为罕见的恶性肿瘤,起源于脑或脑膜的血管内皮细胞。目前对于这种疾病的临床表现、特征和预后还没有很好的认识。在此我们报告一例这样的病例。

病例描述

一名 68 岁的中国男性,表现为意识模糊和右侧肢体运动逐渐障碍。磁共振成像(MRI)显示左额叶有一个出血性病变。手术探查发现一个血肿样病变和广泛的表浅含铁血黄素沉积。术后病理诊断为血管肉瘤。术后 1 个月肿瘤复发,左半球有广泛的肿瘤复发性生长。4 周后患者死亡。

结论

尽管术前准确诊断脑血管肉瘤较为困难,但放射科医生和神经外科医生需要了解这种罕见的实体瘤。颅内血管肉瘤的预后具有异质性。术中广泛的表浅含铁血黄素沉积可能有助于诊断。

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