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颈髓交界处髓内神经鞘瘤。病例报告。

Intramedullary neuroma of the cervicomedullary junction. Case report.

作者信息

Santagata Sandro, Tuli Sagun, Wiese Don E, Day Arthur, De Girolami Umberto

机构信息

Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts 02130, USA.

出版信息

J Neurosurg Spine. 2006 Oct;5(4):362-6. doi: 10.3171/spi.2006.5.4.362.

Abstract

Neuromas typically arise in the peripheral nervous system in response to traumatic injury at the site of partial or complete nerve transection as new axons from the proximal nerve stump sprout to reinnervate the distal segment. In rare cases neuromas have also been described as intramedullary spinal cord lesions. These lesions have been identified as incidental autopsy findings in association with prior trauma and cervical spondylosis, multiple sclerosis, spinal tumors, and syringomyelia. The authors report the case of a 50-year-old man who had been involved in a motor vehicle accident, during which his car was struck from behind as it was stationary at an intersection, more than 5 years before presentation. A workup for syncopal and presyncopal episodes involved magnetic resonance imaging that revealed a 1.1-cm lesion at the cervicomedullary junction (CMJ). The imaging features of the lesion raised the question of an ependymoma or subependymoma. The lesion was excised, and examination of the tissue demonstrated a neuroma with haphazardly arranged interlacing bundles of axons ensheathed by Schwann cells with interfascicular regions of reactive glial cells and Rosenthal fibers, consistent with those present after traumatic injury. This case may represent the first true traumatic intramedullary neuroma of the CMJ diagnosed in a living patient and treated surgically.

摘要

神经瘤通常发生于周围神经系统,是对部分或完全性神经横断部位的创伤性损伤的反应,因为来自近端神经残端的新轴突发芽以重新支配远端节段。在罕见情况下,神经瘤也被描述为脊髓内病变。这些病变已被确定为与既往创伤、颈椎病、多发性硬化症、脊髓肿瘤和脊髓空洞症相关的偶然尸检发现。作者报告了一例50岁男性病例,该患者在就诊前5年多曾发生机动车事故,当时他的车在十字路口静止时被从后面撞上。针对晕厥和晕厥前期发作的检查包括磁共振成像,结果显示在颈髓交界处(CMJ)有一个1.1厘米的病变。该病变的影像学特征引发了室管膜瘤或室管膜下瘤的疑问。病变被切除,组织检查显示为神经瘤,有杂乱排列的交织轴突束,被施万细胞包裹,伴有反应性胶质细胞和罗森塔尔纤维的束间区域,与创伤性损伤后的表现一致。该病例可能代表了首例在活体患者中诊断并接受手术治疗的真正的CMJ创伤性脊髓内神经瘤。

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