摘要
目的探讨血管母细胞瘤临床病理学特征。方法收集50例血管母细胞瘤,分析其临床特点,观察其病理组织学形态及免疫表型特征。结果 50例血管母细胞瘤中男性27例,女性23例,男︰女为1.17︰1.00,平均年龄37.7岁。病变位于小脑者41例(小脑半球28例,小脑蚓部13例),其余9例分别位于脊髓4例,四脑室1例,颞叶1例,额叶1例,枕叶1例,中脑1例。临床表现主要为头晕、头痛、呕吐、视乳头水肿、共济失调等。病理检查:肿瘤主要由不同成熟阶段的毛细血管和毛细血管网之间丰富的间质细胞组成。免疫标记显示:肿瘤组织中的血管内皮细胞均表达CD34和第八因子抗原(FⅧRag),多数间质细胞表达inhibin-a、D2-40,少数表达S-100、神经元特异性烯醇化酶(NSE),血管内皮细胞和间质细胞均表达波形蛋白(Vim),而一般不表达胶质纤维酸性蛋白(GFAP)、上皮膜抗原(EMA)、CK。Ki-67表达较低或不表达。结论血管母细胞瘤主要由不同成熟阶段的毛细血管和来源未定的肿瘤性间质细胞构成。其诊断主要依据病理形态学特征。血管母细胞瘤应注意与转移性肾癌、血管瘤型脑膜瘤和毛细胞型星形细胞瘤相鉴别。
Objective To investigate the clinicopathological features of hemangioblastoma.Methods The clinicopathological features of 50 cases of hemangioblastoma were analyzed.Immunohistochemical study was carried out and literature was reviewed.Results There were 27 males and 23 females.The average age of patients was 37.7 years old.The tumor was located in the cerebellum with 41 cases,in the spinal cord with 4 cases,in the fourth ventrical with 1 case,temporal lobe with 1 cases,in frontal lobe with 1 case,in occipital lobe with 1 case and in midbrain with 1 case.The clinical symptoms of these cases were dizziness,headache,vomitting,optic disc edema and ataxia.Histopathologically,this tumor was characterized by two main components： capillary and stromal cells.Immunohistochemically,the endotheliocyte was positive for CD34 and F Ⅷ RAg,but most of the stromal cells were positive for a-inhibin、D2-40 and part of the cells were also positive for S-100,NSE.The endotheliocyte and the stromal cell were all positive for vimentin,but negative for GFAP,EMA,CK.The expression of Ki-67 was very low.Conclusion Hemangioblastoma is characterized by stromal cells and numerous capillary,but the origin of the stromal cell is not clear.Pathological features were the golden standard for the diagnosis.It needs to be distinguished from renal carcinoma,angiomatous meningioma and pilocytic astrocytoma.
出处
《新疆医科大学学报》
CAS
2010年第11期1307-1309,共3页
Journal of Xinjiang Medical University
关键词
血管母细胞瘤
病理特征
鉴别诊断
免疫组织化学
hemangioblastoma
pathological feature
differential diagonosis
Immunohistochemistry
