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

详细信息    查看全文

• 作者：Marek Prokopienko ; Przemysław Kunert ; ^{pkunert@wp.pl" class="auth_mail" title="E-mail the corresponding author} ; Anna Podgó ; rska ; Andrzej Marchel
• 关键词：vHLS ; von Hippel&ndash ; Lindau syndrome ; IH ; intramedullary hemangioblastoma ; sIH ; sporadic intramedullary hemangioblastoma ; mMS ; modified McCormick scale
• 刊名：Neurologia i Neurochirurgia Polska
• 出版年：2016
• 出版时间：September-October 2016
• 年：2016
• 卷：50
• 期：5
• 页码：349-355
• 全文大小：633 K

文摘

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.