Pothe H, Ritter H, Schneider J
Zentralbl Neurochir. 1983;44(4):335-42.
The mainly infratentorially occurring angioblastomas belong to the group of benign vascular tumours. Histologically, however, one can distinguish from them pleomorphically undifferentiated forms which have an unfavourable prognosis. The article reports on a group of 20 patients who were operated on in the period from 1978 to 1982. The frequency among the brain tumours seen in the same period of time was 4.5 per cent, only related to the infratentorial tumours. Symptomatically, the signs of occlusive hydrocephalus were well to the fore. The exactness of the topical diagnosis by computer tomography in conjunction with microsurgical operation techniques enables a reduction of the operation risk. The postoperative lethality rate was 3 among 20 patients. Attention was drawn to the observation of a family with a genuine v. Hippel-Lindau syndrome in an incomplete form in a descending line with probably autosomal, dominant heredity.
主要发生在幕下的血管母细胞瘤属于良性血管肿瘤组。然而,从组织学上看,可与它们区分出具有不良预后的多形性未分化形式。本文报道了一组在1978年至1982年期间接受手术的20例患者。在同一时期所见的脑肿瘤中,其发生率为4.5%,仅与幕下肿瘤有关。症状上,梗阻性脑积水的体征非常明显。计算机断层扫描结合显微外科手术技术进行局部诊断的准确性能够降低手术风险。20例患者中有3例术后死亡率。注意到观察到一个家族中存在不完全形式的真正的希佩尔-林道综合征,呈下行遗传,可能为常染色体显性遗传。