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马尾部脊索瘤:临床特征和长期手术结果。

Hemangioblastomas of the cauda equina: Clinical features and long-term surgical outcomes.

机构信息

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Fengtai District, Beijing, China.

National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 100021 Beijing, China.

出版信息

Neurochirurgie. 2024 Jan;70(1):101513. doi: 10.1016/j.neuchi.2023.101513. Epub 2023 Nov 4.

Abstract

BACKGROUND

Spinal hemangioblastomas (HBs) that involving cauda equina are rare. Data on clinical characteristics and long-term intervention outcomes of patients harboring cauda equina HBs remain lacking due to its scarcity.

OBJECTIVE

This study aims to present the clinical-radiological features and treatment results of this rare pathology by using cases from a single center.

METHODS

A review of demographic data and intervention outcomes of patients harboring cauda equina HBs in our department between 2009 and 2020 was retrospectively carried out.

RESULTS

Ten consecutive adult patients were incorporated, with a slight female predominance (n = 6, 60%). The mean age was 39.9 ± 14.7 (range: 18-58) years. Six patients (60%) had von Hippel‒Lindau (VHL) syndrome and showed multiple symptoms and severe neurological deficits, while 4 (40%) were in the sporadic group and only presented pain symptoms. During follow-up, 3 patients (30%) experienced lesion relapse and underwent repeated surgery. Favorable outcomes were achieved in all patients.

CONCLUSION

Cauda equina HBs are rare spinal vascular lesions that should be differentiated from other lumbar canal lesions. Total surgical resection is the main treatment modality and can benefit patients, even recurrent patients. The treatment outcome is usually satisfactory, especially in sporadic cases.

摘要

背景

累及马尾的脊髓血管母细胞瘤(HBs)较为罕见。由于其罕见性,目前关于携带马尾 HB 的患者的临床特征和长期干预结果的数据仍然缺乏。

目的

本研究旨在通过单中心病例报告,介绍这种罕见病变的临床-放射学特征和治疗结果。

方法

回顾性分析了 2009 年至 2020 年我科收治的 10 例马尾 HB 患者的人口统计学数据和干预结果。

结果

纳入 10 例连续成年患者,女性略占优势(n=6,60%)。平均年龄为 39.9±14.7(范围:18-58)岁。6 例(60%)患者患有 von Hippel-Lindau(VHL)综合征,表现为多种症状和严重的神经功能缺损,而 4 例(40%)为散发性,仅表现为疼痛症状。在随访期间,3 例(30%)患者出现病变复发并接受了再次手术。所有患者均获得了良好的结局。

结论

马尾 HB 是罕见的脊髓血管病变,应与其他腰椎管病变相鉴别。完全手术切除是主要的治疗方式,可使患者受益,即使是复发病例也是如此。治疗结果通常令人满意,尤其是在散发性病例中。

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