Department of Orthopedic Surgery, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita 010-8543, Japan.
Spine J. 2010 Apr;10(4):e11-5. doi: 10.1016/j.spinee.2010.01.023.
Ganglioneuromas are rarely located in the cervical region compressing the spinal cord. Only two cases of bilateral and symmetric dumbbell tumor have been reported previously.
The purpose of the study was to present an additional case with bilateral and symmetric dumbbell ganglioneuromas of the cervical spine as part of multiple ganglioneuromas of the spine in a patient with neurofibromatosis type 1 (NF-1).
The study design was a case report.
A 15-year-old boy with NF-1 presented with a 6-month history of progressive tetraparesis. Magnetic resonance imaging showed voluminous bilateral and symmetric dumbbell masses at the C1-C2 level severely compressing the spinal cord. The spinal cord was also indented by a dumbbell mass at the left C3-C4 level. A systemic imaging survey of the patient showed numerous asymptomatic foraminal and extraforaminal tumors at all neuroforamina of the spine.
The result was found to be surgical decompression of the spinal cord by subtotal resections of bilateral tumors at the C1-C2 level and unilateral tumor at the left C3-C4 level alleviated patient symptoms. Histopathological diagnosis was ganglioneuroma for all resected tumors.
Multiple ganglioneuromas, particularly bilateral and symmetric dumbbell tumors, are extremely rare but could be associated with NF-1.
神经节细胞瘤很少位于颈椎区域压迫脊髓。以前仅报道过两例双侧和对称哑铃状肿瘤的病例。
本研究的目的是报告一例额外的双侧和对称哑铃状颈段神经节细胞瘤病例，该病例是神经纤维瘤病 1 型（NF-1）患者脊柱多发性神经节细胞瘤的一部分。
研究设计为病例报告。
一名 15 岁男孩患有 NF-1，表现为进行性四肢瘫痪 6 个月。磁共振成像显示 C1-C2 水平有大量双侧和对称哑铃状肿块，严重压迫脊髓。脊髓还被左 C3-C4 水平的哑铃状肿块压陷。对患者进行全身影像学检查显示脊柱所有神经孔的多个无症状椎间孔和椎间孔外肿瘤。
发现通过对 C1-C2 水平的双侧肿瘤和左 C3-C4 水平的单侧肿瘤进行次全切除术来对脊髓进行手术减压，缓解了患者的症状。所有切除的肿瘤的组织病理学诊断均为神经节细胞瘤。
多发性神经节细胞瘤，特别是双侧和对称哑铃状肿瘤，极为罕见，但可能与 NF-1 有关。
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