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小脑血管母细胞瘤(HGB)呈家族发病者少见,连同本文,日本迄今已有7个家族患本病的病例报导。其临床特征为遗传性、其它器管的囊肿、红细胞增多症及视网膜血管瘤(后两者本文报导病例均缺如)。本病约占颅内 HGB 之10%左右,发病年龄以30~39岁最多,然而其下一代患者的发病年龄往往提前。本文作者报导一家族中有3例患者,具体情况如下。例一,27岁,女性,因头痛、呕吐及站立不稳而入院。神经系统检查有小脑受累体征。右椎动脉造影,见右侧小脑内有一由小脑上动脉供养的小指头大小之肿瘤染色。CT 见右小脑有一大的低密度区,其后端正中有一结节状的密度增强区紧邻枕骨。
Cerebellar hemangioblastoma (HGB) is a rare case of familial oncology. Together with this article, 7 cases of this disease have been reported in Japan so far. Its clinical features are hereditary, other device cysts, polycythemia, and retinal hemangiomas (these two cases are reported in the absence of this article). The disease accounts for about 10% of the intracranial HGB, and the age of onset is 30 to 39 years old. However, the age of onset of the next generation of patients is often advanced. The authors report three patients in a family, as follows. For example, at the age of 27, females were admitted to hospital because of headache, vomiting and unstable posture. Neurological examination has signs of cerebellar involvement. Right vertebral arteriography, see the right cerebellum stained by a small finger-sized tumor that is supported by the superior cerebellar artery. The CT shows that the right cerebellum has a large low-density area, with a nodular density-enhancing area in the posterior middle and adjacent to the occipital bone.