Jagannatha Aniruddha Tekkatte, Joshi Krishna Chaitanya, Rao Shilpa, Srikantha Umesh, Varma Ravi Gopal, Mahadevan Anita
Department of Neurosurgery, M S Ramaiah Institute of Neurosciences, M S Ramaiah Medical College, Bengaluru, Karnataka, India.
Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
J Pediatr Neurosci. 2016 Oct-Dec;11(4):319-321. doi: 10.4103/1817-1745.199474.
Spinal schwannomas are commonly intradural extramedullary in location. As Schwann cells are not common in the central nervous system, intramedullary schwannomas are a rare entity. In adults, an estimated sixty cases have been reported in English literature. They are rarer in children (less than ten cases), and preoperative diagnosis becomes a prerogative in achieving total excision. Cervical cord is a common location and less commonly they occur in the conus. We report a rare case of calcified conus intramedullary schwannoma in a child without neurofibromatosis, who presented with conus-cauda syndrome of 1-year duration. Literature has been reviewed regarding its origin, pathophysiology, radiological features, and surgical management. This child underwent laminotomy and subtotal resection of the lesion. Histopathologically, tumor had typical features of schwannoma and was positive for S-100 immunoperoxidase. We believe that schwannoma needs to be considered in the preoperative differential diagnosis of a conus tumor in children as complete excision is possible in these benign tumors, thus affecting a cure.
脊髓神经鞘瘤通常位于硬膜内髓外。由于雪旺细胞在中枢神经系统中并不常见,髓内神经鞘瘤是一种罕见的实体。在成人中,英文文献中估计已报道了60例。它们在儿童中更为罕见(少于10例),术前诊断成为实现完全切除的先决条件。颈髓是常见部位,较少发生在圆锥。我们报告了1例无神经纤维瘤病的儿童圆锥髓内钙化神经鞘瘤罕见病例,该患儿表现为持续1年的圆锥马尾综合征。本文就其起源、病理生理学、影像学特征及手术治疗进行了文献综述。该患儿接受了椎板切开术及病变次全切除术。组织病理学检查显示,肿瘤具有神经鞘瘤的典型特征,S-100免疫过氧化物酶染色呈阳性。我们认为,在儿童圆锥肿瘤的术前鉴别诊断中需要考虑神经鞘瘤,因为这些良性肿瘤有可能实现完全切除,从而实现治愈。