Niğde Ömer Halisdemir University Training and Research Hospital, Niğde, Turkey.
Department of Neurosurgery, Fırat University, Elazığ, Turkey.
Niger J Clin Pract. 2022 Feb;25(2):197-199. doi: 10.4103/njcp.njcp_79_21.
In patients with type 1 neurofibromatosis (NF1), there is an increased susceptibility to tumor development in the central nervous system due to the loss of neurofibromin, an inactivator of the protooncogene Ras. NF1 has a broad clinical spectrum,which includes spinal tumors. Although the most common intramedullary tumor of the spinal cord in adults is ependymoma, few patients with NF1 accompanied by spinal ependymoma have been reported to date, and the localization of the tumors is cervical and thoracic in these cases. In this study, we report the case of a patient with NF1 presenting to our clinic with low back pain and gait disturbance. The patient had an intradural extramedullary ependymoma at the L2-3 vertebra level. This report is the first case of NF1 with spinal ependymoma localized in the lumbar region.
在 1 型神经纤维瘤病(NF1)患者中,由于神经纤维瘤蛋白的缺失,导致原癌基因 Ras 的失活,中枢神经系统肿瘤的易感性增加。NF1 具有广泛的临床谱,包括脊髓肿瘤。尽管成人脊髓最常见的髓内肿瘤是室管膜瘤,但迄今为止,伴有脊髓室管膜瘤的 NF1 患者报道较少,且这些病例中的肿瘤定位于颈胸段。在本研究中,我们报告了 1 例 NF1 患者因腰痛和步态障碍就诊于我院。患者 L2-3 椎体水平存在硬脊膜外髓内室管膜瘤。本报告是首例 NF1 合并位于腰椎区域的脊髓室管膜瘤的病例。