Department of Neurosurgery, Pontchaillou University Hospital, 35033, Rennes cedex 09, France.
Acta Neurochir (Wien). 2013 Jun;155(6):1003-9; discussion 1009. doi: 10.1007/s00701-013-1681-5. Epub 2013 Apr 5.
Haemangioblastomas are benign vascular tumours that may appear sporadically or in von Hippel-Lindau disease. Despite their higher incidence, sporadic haemangioblastomas have been less studied than syndromic ones. In this article, we evaluate the specific features, outcome and quality of life of patients with intracranial sporadic haemangioblastomas (ISHs) operated on in our institution.
Between 1998 and 2010, 38 patients harbouring 38 ISHs were operated on in our department. Their clinical, biological, radiological and surgical features were retrospectively reviewed. All patients were contacted for a quality-of-life (QOL) survey assessed by the Short Form 36 questionnaire (SF36). The mean duration of follow-up was 40 months (13-108 months).
ISH represented 0.9 % of primary intracranial neoplasms treated in our centre during this period. Patients comprised 23 men and 15 women with a mean age of 47 years. None had polycythaemia. Cerebellar locations accounted for 79 % of ISHs, and brainstem ISH with involvement of the floor of the fourth ventricle represented 11 % of ISHs. At last follow-up, two patients harbouring solid medulla oblongata haemangioblastoma had died following severe bulbar syndrome and five patients had died of unrelated causes. One patient had multiple surgeries for three recurrences. Tumoral control was achieved in all cases at last follow-up. Results of the SF-36 questionnaire were as follows: median physical functioning score 100 (range 0-100), median physical problems score 100 (range 0-100), median bodily pain score 100 (range 45-100), median social functioning score 100 (range 25-100), median general mental health score 84 (range 40-92), median emotional problems score 100 (range 0-100), median vitality score 70 (range 35-80) and median general health perceptions score 70 (range 35-100). Mean QOL scores were similar to the general healthy population.
Surgery of ISH provides good QOL and tumoral control except for those located in the medulla oblongata. We recommend considering a careful multimodal therapeutic approach, including radiosurgery for these specific locations.
血管母细胞瘤是良性血管肿瘤，可散发性出现或在 von Hippel-Lindau 病中出现。尽管其发病率较高，但散发性血管母细胞瘤的研究不如综合征性血管母细胞瘤多。本文评估了在我院接受治疗的颅内散发性血管母细胞瘤（ISH）患者的特定特征、结果和生活质量。
1998 年至 2010 年期间，我院共对 38 例 38 例 ISH 患者进行了手术治疗。回顾性分析了其临床、生物学、影像学和手术特征。所有患者均接受了生活质量（QOL）调查，采用 36 项简明健康调查问卷（SF36）进行评估。平均随访时间为 40 个月（13-108 个月）。
在该时期，ISH 占我院治疗的原发性颅内肿瘤的 0.9%。患者包括 23 名男性和 15 名女性，平均年龄为 47 岁。无红细胞增多症。小脑部位占 ISH 的 79%，脑干 ISH 累及第四脑室底部占 11%。末次随访时，2 例实质性延髓长血管母细胞瘤患者因严重延髓综合征死亡，5 例患者因其他原因死亡。1 例患者因 3 次复发接受了多次手术。所有患者的肿瘤均得到控制。SF-36 问卷的结果如下：躯体功能评分中位数 100（范围 0-100），躯体问题评分中位数 100（范围 0-100），身体疼痛评分中位数 100（范围 45-100），社会功能评分中位数 100（范围 25-100），一般心理健康评分中位数 84（范围 40-92），情绪问题评分中位数 100（范围 0-100），活力评分中位数 70（范围 35-80），一般健康感知评分中位数 70（范围 35-100）。平均 QOL 评分与一般健康人群相似。
除位于延髓的肿瘤外，ISH 的手术可提供良好的 QOL 和肿瘤控制。我们建议对这些特定部位考虑谨慎的多模式治疗方法，包括放射外科治疗。
