Object. The majority of intramedullary spinal cord tumors (IMSCT) in children and young adults are low-grade gliomas. Radical resection of similar tumors in the cerebral hemisphere or cerebellum is usually curative; however, the conventional management for IMSCTs remains partial resection followed by radiotherapy because of the concern for surgical morbidity. Nevertheless, radical resection of IMSCTs without routine adjuvant treatment has been the rule at our institution since 1980. In an attempt to resolve this controversy, the long-term morbidity and survival in a large series of children have been retrospectively reviewed. Methods. The database records and current status of 164 patients 21 years of age and younger in whom an IMSCT was resected were reviewed. A gross-total resection (> 95%) was achieved in 76.8% of the surgical procedures. Subtotal resections (80–95%) were performed in 20.1%. The majority of patients (79.3%) had histologically low-grade lesions. There were no deaths due to surgery. When comparing the preoperative and 3-month postoperative functional grades, 60.4% stayed the same, 15.8% improved, and 23.8% deteriorated. Only 13 patients deteriorated by more than one functional grade. Patients with either no deficits or only mild deficits before surgery were rarely injured by the procedure, reinforcing the importance of early diagnosis and treatment. The major determinant of long-term patient survival was histological composition of the tumor. The 5-year progression-free survival rate was 78% for patients with low-grade gliomas and 30% for those with high-grade gliomas. Patients in whom an IMSCT was only partially resected (< 80%) fared significantly worse. Conclusions. The long-term survival and quality of life for patients with low-grade gliomas treated by radical resection alone is comparable or superior to minimal resection and radiotherapy. The optimum therapy for patients with high-grade gliomas is yet to be determined.

Resection of intramedullary spinal cord tumors carries a high risk for surgical damage to the motor pathways. This surgery is therefore optimal for testing the performance of intraoperative motor evoked potential (MEP) monitoring. This report attempts to provide evidence for the accurate representation of patients' pre- and postoperative motor status by combined epidural and muscle MEP monitoring during intramedullary surgery. The authors used transcranial electrical motor cortex stimulation to elicit MEPs, which were recorded from the spinal cord (with an epidural electrode) and from limb target muscles (thenar, anterior tibial) with needle electrodes. The amplitude of the epidural MEPs and the presence or absence of muscle MEPs were the parameters for MEP interpretation. A retrospective analysis was performed on data from the resection of 100 consecutive intramedullary tumors and MEP data were compared with the pre- and postoperative motor status. Intraoperative monitoring was feasible in all patients without severe preoperative motor deficits. Preoperatively paraplegic patients had no recordable MEPs. The sensitivity of muscle MEPs to detect postoperative motor deficits was 100% and its specificity was 91%. There was no instance in which a patient with stable MEPs developed a motor deficit postoperatively. Intraoperative MEPs adequately represented the motor status of patients undergoing surgery for intramedullary tumors. Because deterioration of the motor status was transient in all cases, it can be considered that impairment of the functional integrity of the motor pathways was detected before permanent deficits occurred.

Thirty-eight patients underwent surgery for an intramedullary spinal cord ependymoma. In 37 patients, postoperative magnetic resonance imaging confirmed that the tumor was totally removed. The morbidity of surgery was directly related to the preoperative neurological condition. Patients who were normal or nearly normal preoperatively were rarely worse after surgery, and those who had significant disability preoperatively were at greatest risk of being more impaired after surgery. There has been no tumor recurrence in any patient after a mean follow-up period of 24 months, and radiation therapy has not been employed as a surgical adjunct.

The authors review their experience with the operative management of 19 consecutive cases of malignant astrocytoma of the spinal cord. There was a male to female ratio of 1.1:1, and the median age of the population was 14 years (range 1 to 32 years). The median duration of symptoms prior to definitive diagnosis was 7 weeks. Radical excision was carried out in all cases, with 18 patients (95%) receiving radiotherapy and 10 patients (53%) receiving chemotherapy as well. To date, 15 (79%) of the 19 patients in this series have died, with a median survival period of 6 months following surgery. No patient improved after operation. Hydrocephalus was present in 11 patients (58%), seven of whom underwent ventricular shunting procedures. Dissemination of disease was found in 11 patients (58%). Extraneural metastases did not occur in the absence of a ventricular shunt. The authors conclude that malignant astrocytomas of the spinal cord are heralded by a short history followed by rapid neurological deterioration and usually death. The rationale for operation is discussed, and an aggressive approach utilizing adjuvant therapy directed at the entire neuraxis is suggested.