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圆锥髓质水平的硬脊膜内、髓外血管母细胞瘤:病例展示

Intradural, extramedullary hemangioblastoma at the level of the conus medullaris: illustrative case.

作者信息

Shields Lisa B E, Harpring John E, Highfield Hilary A, Zhang Yi Ping, Shields Christopher B

机构信息

Norton Neuroscience Institute and.

CPA Laboratory, Norton Healthcare, Louisville, Kentucky; and.

出版信息

J Neurosurg Case Lessons. 2021 Apr 26;1(17):CASE2145. doi: 10.3171/CASE2145.

Abstract

BACKGROUND

Hemangioblastomas are rare, slow-growing, and highly vascularized tumors that typically occur in the cerebellum and spinal cord. The cervical and thoracic regions are the most common spinal sites, and the tumors are usually intramedullary.

OBSERVATIONS

The authors report the case of a man whose chest computed tomography performed for managing coronavirus disease 2019 incidentally revealed an enhancing area in the spinal canal at T12-L1. The patient reported low back pain as well as leg numbness and tingling. Magnetic resonance imaging of the lumbar region with and without gadolinium contrast demonstrated an intradural, extramedullary lesion with displacement of the conus medullaris. The differential diagnosis included a schwannoma and myxopapillary ependymoma. Bilateral T12-L1 laminectomies were performed with resection of the mass. The general pathologist rendered the frozen section diagnosis of a spindle cell neoplasm, suggesting the differential diagnosis of schwannoma or myxopapillary ependymoma. Immunohistochemistry was positive for inhibin, GFAP, reticulin, CD31, , S100, and EMA. A World Health Organization grade 1 hemangioblastoma was confirmed.

LESSONS

Spinal surgeons should be cognizant of the presenting symptoms and differential diagnosis of hemangioblastomas at the level of the conus medullaris, especially when the tumor is diagnosed incidentally. Additional investigations should be performed to determine whether von Hippel-Lindau syndrome is associated with the hemangioblastoma, as this combination portends a different clinical presentation, multiple tumor locations, and tumor recurrence following resection.

摘要

背景

血管母细胞瘤是一种罕见的、生长缓慢且血管高度丰富的肿瘤,通常发生于小脑和脊髓。颈椎和胸椎区域是脊髓最常见的发病部位,且肿瘤通常位于髓内。

观察结果

作者报告了一例男性病例,其因管理2019冠状病毒病而进行的胸部计算机断层扫描偶然发现T12-L1椎管内有一个强化区域。患者自述下背部疼痛以及腿部麻木和刺痛。腰椎区域钆对比剂增强前后的磁共振成像显示硬膜内、髓外病变,伴圆锥马尾移位。鉴别诊断包括神经鞘瘤和黏液乳头型室管膜瘤。行双侧T12-L1椎板切除术并切除肿物。病理科普通医师对冰冻切片诊断为梭形细胞瘤,提示神经鞘瘤或黏液乳头型室管膜瘤的鉴别诊断。免疫组化结果显示抑制素、胶质纤维酸性蛋白、网硬蛋白、CD31、S100和上皮膜抗原呈阳性。确诊为世界卫生组织1级血管母细胞瘤。

经验教训

脊柱外科医生应认识到圆锥马尾水平血管母细胞瘤的症状表现和鉴别诊断,尤其是肿瘤为偶然诊断时。应进行进一步检查以确定血管母细胞瘤是否与冯·希佩尔-林道综合征相关,因为这种组合预示着不同的临床表现、多个肿瘤部位以及切除后肿瘤复发。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d783/9245781/de4e5f93db27/CASE2145f1.jpg

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