Kim Na Rae, Suh Yeon-Lim, Shin Hyung-Jin
Department of Pathology, Gil Medical Center, Gachon University of Medicine and Science, Incheon, Korea.
Pediatr Neurosurg. 2009;45(5):396-401. doi: 10.1159/000260911. Epub 2009 Nov 26.
Schwann cells are generally present in the nerve root, not in the spinal cord. Therefore, intramedullary schwannomas are rare and correct preoperative diagnosis is often difficult. To date, less than 50 cases of intramedullary schwannoma with no sign of neurofibromatosis have been reported in the English literature, of which 5 occurred in children. Here, we report a case of primary thoracic intramedullary schwannoma which occurred at the age of 11, confirmed by intraoperative frozen biopsy. The patient presented with decreased sensation below the T6 dermatome at the right side and right lower extremity weakness with urge incontinence. The ideal treatment for intramedullary schwannoma of histologically benign nature is surgical resection. However, the mass was subtotally resected due to unfavorable intraoperative somatosensory-evoked potential findings, and postoperative fractionated radiotherapy followed. She has been alive with the residual intramedullary mass with no discernible changes during the 11.5 years of follow-up. When neurosurgeons, pediatricians and radiologists encounter an intramedullary tumor in a pediatric patient, intramedullary schwannoma should be included in the differential diagnoses because it might be curable if excised.
施万细胞通常存在于神经根,而非脊髓。因此,髓内神经鞘瘤较为罕见,术前准确诊断往往困难。迄今为止,英文文献报道的无神经纤维瘤病迹象的髓内神经鞘瘤病例不足50例,其中5例发生于儿童。在此,我们报告一例11岁原发性胸段髓内神经鞘瘤病例,术中冰冻活检得以确诊。患者表现为右侧T6皮节以下感觉减退、右下肢无力及急迫性尿失禁。对于组织学性质为良性的髓内神经鞘瘤,理想的治疗方法是手术切除。然而,由于术中体感诱发电位结果不佳,肿块仅行次全切除,术后进行了分次放疗。在11.5年的随访期间,她带着残留的髓内肿块存活,未见明显变化。当神经外科医生、儿科医生和放射科医生遇到儿科患者的髓内肿瘤时,应将髓内神经鞘瘤纳入鉴别诊断,因为如果切除,它可能是可治愈的。