BACKGROUND AND PURPOSE: Intramedullary hemangioblastomas are relatively rare intraspinal tumors. Total removal of these tumors without causing significant neurological deficit remains a great challenge. This study analyzed the preoperative characteristics, management, and outcome in patients with successful total removal of spinal intramedullary hemangioblastomas. METHODS: Data from the medical records of patients with intraspinal hemangioblastomas treated from 1993 to 2003 were reviewed. The neurological function of these patients was graded and the preoperative data were recorded and correlated with clinical outcome. RESULTS: Ten patients with spinal intramedullary hemangioblastomas underwent microsurgical resection by the same neurosurgeon during the 10-year study period. They included 3 men and 7 women with age ranging from 20 to 49 years, with a mean age of 33 years. Five of the patients met the diagnostic criteria for von Hippel-Lindau disease. Preoperative neurological function was grade I in 6 patients, grade II in 3 patients and grade III in 1 patient. Immediate postoperative neurological function was worse in 3 cases, but all of these patients recovered their preoperative function within 3 weeks. At 3 months' follow-up, 9 patients had achieved functional grade I, and 1 patient with preoperative grade III function had improved to grade II. CONCLUSIONS: Detailed preoperative evaluation and skillful microsurgery are mandatory in successful total removal of intramedullary hemangioblastomas. The management of spinal hemangioblastomas associated with von Hippel-Lindau disease requires a cautious observation of clinical course and timely surgical intervention of symptomatic lesions to avoid possible neurological deficit.
BACKGROUND AND PURPOSE: Intramedullary hemangioblastomas are relatively rare intraspinal tumors. Total removal of these tumors without causing significant neurological deficit remains a great challenge. This study analyzed the preoperative characteristics, management, and outcome in patients with successful total removal of spinal intramedullary hemangioblastomas. METHODS: Data from the medical records of patients with intraspinal hemangioblastomas treated from 1993 to 2003 were reviewed. The neurological function of these patients was graded and the preoperative data were recorded and correlated with clinical outcome. RESULTS: Ten patients with spinal intramedullary hemangioblastomas underwent microsurgical resection by the same neurosurgeon during the 10-year study period. They included 3 men and 7 women with age ranging from 20 to 49 years, with a mean age of 33 years. Five of the patients met the diagnostic criteria for von Hippel-Lindau disease. Preoperative neurological function was grade I in 6 patients, grade II in 3 patients and grade III in 1 patient. Immediate postoperative neurological function was worse in 3 cases, but all of these patients recovered their preoperative function within 3 weeks. At 3 months' follow-up, 9 patients had achieved functional grade I, and 1 patient with preoperative grade III function had improved to grade II. CONCLUSIONS: Detailed preoperative evaluation and skillful microsurgery are mandatory in successful total removal of intramedullary hemangioblastomas. The management of spinal hemangioblastomas associated with von Hippel-Lindau disease requires a cautious observation of clinical course and timely surgical intervention of symptomatic lesions to avoid possible neurological deficit.