Arishima Hidetaka, Kitai Ryuhei, Kodera Toshiaki, Yamada Shinsuke, Kikuta Ken-ichiro
Department of Neurosurgery, University of Fukui.
Neurol Med Chir (Tokyo). 2014;54(9):752-6. doi: 10.2176/nmc.cr.2013-0257. Epub 2014 Jan 10.
Neurofibromas are occasionally present in spinal roots; however, an intramedullary neurofibroma is especially rare. Although a few cases of intramedullary neurofibromas in cervical spinal cord have been reported, to the best of our knowledge, there are no reports of intramedullary neurofibromas in thoracic spinal cord, and moreover, no reports have clearly reported immunohistochemical findings. We report a rare case of a large intramedullary neurofibroma in the thoracic spinal cord and show immunohistochemical examination of the tumor. A 52-year-old man presented with a 2-year history of progressive gait disturbance. Neurological examinations demonstrated complete motor and sensory deficit of his legs. Magnetic resonance imaging of the thoracic spine demonstrated an intramedullary enhancing mass within the spinal cord between T4 and T5 levels. The patient underwent T3-T6 laminectomy surgery. The dura mater was opened to reveal fusiform dilatation of the spinal cord and a midline myelotomy was performed. An intramedullary mass was revealed and could be resected totally. Histopathological examination revealed that the tumor cells exhibited spindle-shaped and wavy nuclei with abundant collagen, which resembled schwannoma or fibrous meningioma. By immunohistochemical examination, some tumor cells were positive for S-100 proteins; however, most tumor cells were strongly positive for CD34. From these pathological findings and immunohistochemical reactions, we diagnosed the intramedullary tumor as a neurofibroma.
神经纤维瘤偶尔出现在脊神经根;然而,髓内神经纤维瘤尤其罕见。虽然已有少数颈髓髓内神经纤维瘤的病例报道,但据我们所知,尚无胸髓髓内神经纤维瘤的报道,而且也没有明确报道免疫组化结果的。我们报告一例罕见的胸髓大型髓内神经纤维瘤病例,并展示该肿瘤的免疫组化检查结果。一名52岁男性,有2年进行性步态障碍病史。神经系统检查显示其双下肢完全运动和感觉功能缺失。胸椎磁共振成像显示在T4和T5水平之间的脊髓内有一个髓内强化肿块。患者接受了T3 - T6椎板切除术。打开硬脑膜后发现脊髓呈梭形扩张,并进行了中线脊髓切开术。发现一个髓内肿块并将其完全切除。组织病理学检查显示肿瘤细胞呈梭形,核呈波浪状,有丰富的胶原,类似神经鞘瘤或纤维性脑膜瘤。通过免疫组化检查,一些肿瘤细胞S - 100蛋白呈阳性;然而,大多数肿瘤细胞CD34呈强阳性。根据这些病理结果和免疫组化反应,我们将髓内肿瘤诊断为神经纤维瘤。