Serban D, Exergian F
Bagdasar Arseni Clinical Emergency Hospital, Bucharest, Romania.
Chirurgia (Bucur). 2013 May-Jun;108(3):325-30.
Intramedullary hemangioblastomas are rare benign tumors, occurring sporadically or in von Hippel- Lindau disease.
We describe our local surgical experience with intramedullary hemangioblastomas. Clinical, imaging and surgical data from five consecutive hemangioblastoma cases identified from a series of 59 patients with intramedullary tumors treated between 2003-2009 are reviewed.
The mean age of the patients was 39.6 years (range 21- 56). All of them were symptomatic and two patients had von Hippel-Lindau disease with associated posterior fossa hemangioblastomas. All tumors were preoperatively diagnosed as hemangioblastomas based on magnetic resonance findings. All patients underwent surgery with complete removal of the tumor in 4 cases and a partial removal in a case with extension towards the anterior part of the cord. Good neurological outcome was noted in four cases while in the fifth, complicated with a significant intraoperative hemorrhage, a fully reversible aggravation of neurological status occurred.
Spinal cord hemangioblastomas are surgically curable tumors. Microsurgical complete resection is the standard of care and can be performed with good neurological outcome in most of the cases. Ventral tumor location and important intraoperative bleeding are associated with less optimal outcome.
髓内血管母细胞瘤是罕见的良性肿瘤，可散发或发生于冯·希佩尔-林道病。
我们描述了我们在髓内血管母细胞瘤方面的局部手术经验。回顾了从2003年至2009年治疗的59例髓内肿瘤患者中连续5例血管母细胞瘤病例的临床、影像学和手术数据。
患者的平均年龄为39.6岁（范围21至56岁）。他们均有症状，2例患者患有冯·希佩尔-林道病并伴有后颅窝血管母细胞瘤。所有肿瘤术前均根据磁共振成像结果诊断为血管母细胞瘤。所有患者均接受了手术，4例肿瘤完全切除，1例肿瘤向脊髓前部延伸仅部分切除。4例患者神经功能恢复良好，第5例患者术中发生大量出血，神经功能出现完全可逆性恶化。
脊髓血管母细胞瘤是可通过手术治愈的肿瘤。显微外科完全切除是治疗的标准方法，大多数情况下可取得良好的神经功能预后。肿瘤位于腹侧及术中大量出血与预后欠佳相关。
