Kothbauer Karl F
Division of Neurosurgery, Department of Surgery, Kantonsspital Luzern, Postfach, Luzern, Switzerland.
Pediatr Neurosurg. 2007;43(3):222-35. doi: 10.1159/000098835.
The majority of intramedullary spinal cord tumors in children are low-grade glial tumors. They become symptomatic with pain, neurologic deficits or spinal deformity. The diagnosis is most readily obtained using magnetic resonance imaging. The natural history is significant for slow progression of symptoms. Surgery is the best treatment and is also indicated to confirm the histological diagnosis. In case of a low-grade tumor or a vascular lesion such as hemangioblastoma or cavernoma, a total or near-total resection is attempted. For astrocytomas the resection almost always remains biologically incomplete, but a near-total resection is still associated with a long progression-free survival. Neurologic morbidity is relatively low during long-term follow-up but can be up to 30% for transient motor deficits. The risk for neurologic deterioration is higher for patients with pronounced dysfunction preoperatively. This is an important argument for early surgical resection. Surgery is performed using the spectrum of microsurgical techniques as well as advanced technology, e.g. lasers and intraoperative neurophysiological monitoring with motor evoked potentials. High-grade tumors are resected conservatively and treated with radiation and chemotherapy. The prognosis of high-grade glial tumors remains poor.
儿童脊髓髓内肿瘤大多为低级别胶质瘤。它们会因疼痛、神经功能缺损或脊柱畸形而出现症状。通过磁共振成像最容易做出诊断。其自然病程的特点是症状进展缓慢。手术是最佳治疗方法,也用于确诊组织学诊断。对于低级别肿瘤或血管病变,如成血管细胞瘤或海绵状血管瘤,尝试进行全切或近全切。对于星形细胞瘤,几乎总是无法实现生物学上的全切,但近全切仍与较长的无进展生存期相关。长期随访期间神经功能障碍相对较低,但短暂性运动功能缺损的发生率可达30%。术前功能明显障碍的患者神经功能恶化的风险更高。这是早期手术切除的一个重要依据。手术采用一系列显微外科技术以及先进技术,如激光和术中运动诱发电位神经生理监测。高级别肿瘤采用保守切除,并进行放疗和化疗。高级别胶质瘤的预后仍然很差。