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

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.

journal_name

Neurol Neurochir Pol

authors

Prokopienko M,Kunert P,Podgórska A,Marchel A

doi

10.1016/j.pjnns.2016.06.003

subject

Has Abstract

pub_date

2016-01-01 00:00:00

pages

349-55

issue

5

eissn

0028-3843

issn

1897-4260

pii

S0028-3843(16)30056-1

journal_volume

50

pub_type

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