中枢神经的血管母细胞瘤有12～32％伴发红细胞增多症,其发生机制尚不十分明瞭。一、病例报告:患者女性,29岁,入院前三个月开始恶心呕吐、头痛和走路不稳。通过脑室造影,临床诊断为颅后窝占位性病变。术中见右侧小脑半球有一乒乓球大囊肿,囊内积满草黄色液体,囊壁一侧有指顶大紫红色瘤壁结节,与软脑膜粘连。二、观察结果:光镜下为典型血管母细胞瘤组织像。电镜下肿瘤由三种细胞构成。其一是内皮细胞,直接构成毛细血管的内膜,外面紧接是周围细胞,二者均包括一层基膜。其三是间质细胞,处于毛细血管之间,呈多角形,细胞外多不见基膜(左图)。 Hemangioblastoma of the central nervous system has 12 to 32% of erythrocytosis, and its mechanism is not yet clear. I. Case report: The patient, female, was 29 years old and began nausea and vomiting, headache and walking instability three months before admission. Through ventriculography, clinical diagnosis of posterior fossa space-occupying lesions. During the operation, the right cerebellar hemisphere had a big cystic cyst, and the capsule was filled with grassy yellow liquid. There was a large purplish red tumor wall nodule on the side of the capsule wall, which was in contact with the pia mater. Second, the observation results: light microscope for a typical hemangioblastoma tissue. Under the electron microscope, the tumor consists of three types of cells. One is the endothelial cells, which directly form the inner membrane of the capillaries and are surrounded by peripheral cells. Both include a basement membrane. The third is the mesenchymal cells, which are between the capillaries, showing a polygonal shape, and most of the extracellular matrix membranes are not seen (left).