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Surgical treatment of sporadic and von Hippel–Lindau syndrome-associated intramedullary hemangioblastomas
Affiliations- Department of Neurosurgery, Medical University of Warsaw, Banacha 1a, 02-097 Warsaw, Poland
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Abstract
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.
MethodsTwelve 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.
ResultsThe 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.
ConclusionsThe 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.
Abstract
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.
MethodsTwelve 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.
ResultsThe 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.
ConclusionsThe 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.
Keywords
vHLS, IH, sIH, mMS
About this articleTitle
Surgical treatment of sporadic and von Hippel–Lindau syndrome-associated intramedullary hemangioblastomas
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
349-355
Page views
263
Article views/downloads
562
DOI
10.1016/j.pjnns.2016.06.003
Bibliographic record
Neurol Neurochir Pol 2016;50(5):349-355.
Keywords
vHLS
IH
sIH
mMS
Authors
Marek Prokopienko
Przemysław Kunert
Anna Podgórska
Andrzej Marchel
