Hayashi Yutaka, Nakada Mitsutoshi, Mohri Masanao, Murakami Hideki, Kawahara Norio, Hamada Jun-Ichiro
Department of Neurosurgery, Kanazawa University Hospital, Kanazawa, Japan.
Pediatr Neurosurg. 2011;47(3):210-3. doi: 10.1159/000331569. Epub 2011 Oct 27.
Gangliogliomas of the spinal cord are rare, and the conus medullaris is an extremely rare site for their occurrence. The authors present a case in which a ganglioglioma was found in the thoracolumbar spinal cord, including the conus medullaris, of a 5-year-old female patient with neurofibromatosis type 1 (NF1) who presented with paraparesis and urinary disturbance. MRI revealed an intramedullary lesion within the thoracolumbar spinal cord, including the conus medullaris, which was surgically removed. Pathological investigation showed a ganglioglioma consisting of glioneuronal tumor cells. This is the first report to provide a pathological description of a spinal cord ganglioglioma in a patient with NF1. Because gangliogliomas usually have a good prognosis following resection, it is important to clearly distinguish them from other NF1-associated lesions, even though ganglioglioma of the thoracolumbar spinal cord, including the conus medullaris, is an extremely rare condition.
脊髓神经节胶质瘤很罕见，而脊髓圆锥是其发生的极其罕见部位。作者报告了一例病例，一名患有1型神经纤维瘤病（NF1）的5岁女性患者，出现双下肢轻瘫和排尿障碍，在其胸腰段脊髓包括脊髓圆锥发现了神经节胶质瘤。磁共振成像（MRI）显示胸腰段脊髓包括脊髓圆锥内有一髓内病变，该病变已通过手术切除。病理检查显示为一个由神经胶质神经元肿瘤细胞组成的神经节胶质瘤。这是第一份对NF1患者脊髓神经节胶质瘤进行病理描述的报告。由于神经节胶质瘤切除后通常预后良好，因此即使胸腰段脊髓包括脊髓圆锥的神经节胶质瘤极为罕见，将其与其他NF1相关病变清楚区分开来也很重要。
