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表现为硬膜外脊髓病变的转移性血管外皮细胞瘤。

Metastatic hemangiopericytoma presenting as an epidural spinal cord lesion.

作者信息

Brass Steven David, Guiot Marie-Christine, Albrecht Steffen, Glikstein Rafael, Mohr Gérard

机构信息

Department of Neurology, Harvard University, Brigham and Women's Hospital, Boston, MA, USA.

出版信息

Can J Neurol Sci. 2004 Nov;31(4):550-3. doi: 10.1017/s0317167100003796.

Abstract

OBJECTIVE

Hemangiopericytoma is an aggressive vascular tumour that rarely affects the central nervous system and is even more rarely spinal in presentation. The clinical features of a patient with a recurrent extraspinal hemangiopericytoma presenting with an epidural spinal cord compression by local invasion are described, including a review of the literature on metastatic hemangiopericytoma to the spine.

METHODS

A case of a 53-year-old male, with a recurrent extraspinal hemangiopericytoma which metastasized to the thoracic spine five years after detection of the primary tumour is presented. A chart review was conducted where all pertinent history, physical, laboratory, and radiological data were collected. A Pub-Med search using the keyword "hemangiopericytoma" identified all reported cases documenting clinical features, treatment, recurrence and outcome with respect to metastatic hemangiopericytoma to the spine.

RESULTS

Nine patients have been reported to have metastatic hemangiopericytoma to the spine. The median patient age was 47 years and there was a slight male preference. An unusual feature of the hemangiopericytoma is the prolonged period, up to 16 years, between the diagnosis of the primary hemangiopericytoma and the metastases to the spine. All patients were treated with a combination of radiation and surgery.

CONCLUSIONS

Hemangiopericytomas show a slow clinical evolution with a strong propensity to relapse long after previous treatment and thus, once identified, prolonged follow-up for recurrence is indicated. A close follow-up of these patients is required because of frequent recurrences and delayed metastases even if the primary lesion was well-controlled. Although overall uncommon, hemangiopericytoma should be kept in mind in the differential diagnosis of vascular epidural spinal cord tumours.

摘要

目的

血管外皮细胞瘤是一种侵袭性血管肿瘤,很少累及中枢神经系统,脊柱受累更为罕见。本文描述了一例复发性椎体外血管外皮细胞瘤患者,因局部侵犯导致硬膜外脊髓压迫的临床特征,并对脊柱转移性血管外皮细胞瘤的文献进行了综述。

方法

报告一例53岁男性患者,其原发性肿瘤发现五年后,复发性椎体外血管外皮细胞瘤转移至胸椎。进行了病历回顾,收集了所有相关的病史、体格检查、实验室和影像学数据。使用关键词“血管外皮细胞瘤”在PubMed上进行搜索,确定了所有报告的病例,记录了脊柱转移性血管外皮细胞瘤的临床特征、治疗、复发和预后。

结果

已有9例患者被报道患有脊柱转移性血管外皮细胞瘤。患者的中位年龄为47岁,男性略多。血管外皮细胞瘤的一个不寻常特征是,从原发性血管外皮细胞瘤诊断到脊柱转移之间的时间长达16年。所有患者均接受了放疗和手术联合治疗。

结论

血管外皮细胞瘤临床进展缓慢,在先前治疗后很长时间仍有强烈的复发倾向,因此,一旦确诊,应进行长期随访以监测复发情况。由于即使原发性病变得到良好控制,这些患者仍频繁复发且转移延迟,因此需要密切随访。尽管总体上不常见,但在血管性硬膜外脊髓肿瘤的鉴别诊断中应考虑血管外皮细胞瘤。

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