open access

Vol 50, No 5 (2016)
Original research articles
Submitted: 2015-10-01
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Surgical treatment of sporadic and von Hippel–Lindau syndrome-associated intramedullary hemangioblastomas

Marek Prokopienko, Przemysław Kunert, Anna Podgórska, Andrzej Marchel
DOI: 10.1016/j.pjnns.2016.06.003
·
Neurol Neurochir Pol 2016;50(5):349-355.

open access

Vol 50, No 5 (2016)
Original research articles
Submitted: 2015-10-01

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.

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.

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Keywords

vHLS, IH, sIH, mMS

About this article
Title

Surgical treatment of sporadic and von Hippel–Lindau syndrome-associated intramedullary hemangioblastomas

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 50, No 5 (2016)

Pages

349-355

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

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