Marek Prokopienko1, Przemysław Kunert2, Anna Podgórska1, Andrzej Marchel1. 1. Department of Neurosurgery, Medical University of Warsaw, Warsaw, Poland. 2. Department of Neurosurgery, Medical University of Warsaw, Warsaw, Poland. Electronic address: pkunert@wp.pl.
Abstract
OBJECT: Intramedullary hemangioblastomas are rare lesions. They can be related to von Hippel-Lindau syndrome or they may be sporadic. This study describes surgical treatment for this infrequent tumor. METHODS: Twelve consecutive patients received surgery to remove sporadic or von Hippel-Lindau syndrome-associated intramedullary hemangioblastomas. Patients were evaluated at four time points: before treatment, on postoperative day one, on the day of discharge, and at a follow-up examination. RESULTS: The patients showed good preoperative neurological status. The cohort had a slight female predominance. All tumors spanned at least one spinal segment. In all cases, total tumor removal was achieved, and a good outcome was obtained. None of the following factors had a significant effect on outcome: age, sex, tumor size, the presence of a syrinx, or the presence of von Hippel-Lindau syndrome. CONCLUSIONS: The surgical removal of intramedullary hemangioblastomas resulted in satisfactory long-term functional outcomes. The best results were obtained before neurological symptoms occurred. Thus, we suggest that surgery should be considered for managing asymptomatic, surgically accessible, space-occupying lesions in sIH group, and isolated, space-occupying lesions in vHLS-IH group.
OBJECT: Intramedullary hemangioblastomas are rare lesions. They can be related to von Hippel-Lindau syndrome or they may be sporadic. This study describes surgical treatment for this infrequent tumor. METHODS: Twelve consecutive patients received surgery to remove sporadic or von Hippel-Lindau syndrome-associated intramedullary hemangioblastomas. Patients were evaluated at four time points: before treatment, on postoperative day one, on the day of discharge, and at a follow-up examination. RESULTS: The patients showed good preoperative neurological status. The cohort had a slight female predominance. All tumors spanned at least one spinal segment. In all cases, total tumor removal was achieved, and a good outcome was obtained. None of the following factors had a significant effect on outcome: age, sex, tumor size, the presence of a syrinx, or the presence of von Hippel-Lindau syndrome. CONCLUSIONS: The surgical removal of intramedullary hemangioblastomas resulted in satisfactory long-term functional outcomes. The best results were obtained before neurological symptoms occurred. Thus, we suggest that surgery should be considered for managing asymptomatic, surgically accessible, space-occupying lesions in sIH group, and isolated, space-occupying lesions in vHLS-IH group.