论文部分内容阅读
目的探讨脑脊髓血管母细胞瘤的临床病理特征及间质细胞的起源，为该肿瘤的病理诊断和鉴别诊断提供病理形态依据。方法对33例脑脊髓血管母细胞瘤作回顾性临床病理观察，其中8例进行GFAP、Vimmentin、CD34、Mac387．、a－AT、CK免疫组化标记；3例作了电镜观察。结果发病年龄从7～75岁，平均37．9岁；27例发生于小脑，占81．8％，其他在脑干、脊髓、第四脑室占18．2％；22例发生囊性变（66．7％）；有颅内高压症状者26例，占78．79％，共济失调者19例，占57．58％，二者同存者15例，占45．45％；术后复发者4例，占12．12％。肿瘤内间质细胞对GFAP、Pac387、a－AT、CK缺乏阳性表达，而Vimmentin阳性。电镜证实肿瘤细胞具有血管内皮细胞分化特征。结论脑脊髓血管母细胞瘤是一种好发于成人，临床以颅内高压症状为主要特征，其间质细胞具有表达间叶细胞特性的术后易复发的肿瘤。
Objective To investigate the clinicopathological features and the origin of mesenchymal cells in hemangioblastoma of the brain and spinal cord, and provide pathological morphological basis for the pathological diagnosis and differential diagnosis of this tumor. Methods 33 cases of hemangioblastoma of the brain were retrospectively reviewed. 8 cases were treated with GFAP, Vimentin, CD34 and Mac387. , a-AT, CK immunohistochemical markers; 3 cases were observed by electron microscopy. Results The age of onset ranged from 7 to 75 years old with an average of 37.9 years old; 27 cases occurred in the cerebellum, accounting for 81.8%; the others accounted for 18.2% in the brain stem, spinal cord, and fourth ventricle; 22 cases of cystic degeneration ( 66.7%); There were 26 cases of intracranial hypertension, accounting for 78.79%, 19 cases of ataxia, accounting for 57.58%, 15 cases of co-existence of the two, accounting for 45.45%; 4 cases of recurrence, accounting for 12.12%. The tumor interstitial cells lack positive expression of GFAP, Pac387, a-AT, CK, and Vimmentin positive. Electron microscopy confirmed that the tumor cells had vascular endothelial cell differentiation characteristics. Conclusion Cerebrospinal hemangioblastoma is a kind of adult preponderance. The clinical features of intracranial hypertension are the main features. The mesenchymal cells of the hemangioblastoma have the characteristics of mesenchymal cells expressing postoperative recurrence.