目的：探讨 von Hippel-Lindau 综合征中枢神经系统血管母细胞瘤临床病理特征。方法回顾性分析本院自2013年以来收治的10例神经系统血管网状细胞瘤患者的临床资料，总结 von Hippel-Lindau 综合征中枢神经系统血管母细胞瘤临床病理特征。结果巨检可见肿瘤病灶以小脑及延髓多见，囊性改变，大小1～5 cm，平均（3．1±0．2）cm，边界清晰，囊内可见黄色囊液；镜检可见肿瘤病灶内存在丰富的血液供应，存在有内皮细胞增生，于血管间可见增生肥大，排列呈巢状或分叶状的间质细胞，且间质细胞其细胞质较为丰富，淡染，并可见期内含有丰富类脂质，以空泡状或泡沫状为多见。结论 von Hippel-Lindau 综合征以囊性病变多见，镜检可见肿瘤病灶内存在丰富的血液供应，存在有内皮细胞增生，于血管间可见增生肥大等改变。%Objective To investigate the clinicopathological features of central nervous system hemangioblastoma in von Hippel-Lindau syndrome.Methods The data of 10 patients with central nervous system hemangioblastoma in our hospital since 2013 were analyzed retro-spectively,and the clinicopathological features of central nervous system hemangioblastoma in von Hippel-Lindau syndrome were summarized. Results The macroexamination result showed that most tumor lesions were found in the cerebellum and medulla oblongata,with cystic chan-ges,size from 1 cm to 5 cm,the average size was (3.1 ±0.2)cm,clear boundary,intracapsular yellow cyst fluid.The microscopy result showed tumor foci with a rich blood supply,endothelial cell proliferation and hypertrophy in vascular,which arranged in nests or lobulated mesenchymal cells,the cytoplasm of stromal cells was abundant and lightly stained,a rich lipid was seen,with vesicular or foam.Conclusion The von Hippel-Lindau syndrome is usually cystic lesion,the microscopic examination shows tumor foci with rich blood supply,endothelial cell proliferation,hypertrophy and other changes in vascular.
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