摘要
目的探讨血管母细胞瘤的临床病理特征。方法回顾性分析确诊的血管母细胞瘤患者18例,复阅其临床资料及病理切片,并全部做免疫组化检查。结果 18例患者均为散发病例,16例首发,2例复发,首次发病年龄8~61岁。15例肿瘤位于小脑,2例位于脊髓,1例位于桥小脑角。术前影像学均提示占位,镜下肿瘤由不同成熟阶段的毛细血管和间质细胞构成。免疫组织化学法显示毛细血管及间质细胞Vi M均阳性,血管内皮细胞CD34阳性,间质细胞EGFR阳性12例,NSE阳性10例,S-100阳性9例,Ki-67指数较低。结论血管母细胞瘤多见于中青年的小脑和脊髓,但儿童患者并不少见,脊髓及小脑以外的神经系统,比如垂体柄、鞍上、软脑膜及颅神经、桥小脑角也可发病。临床症状与具体病变部位密切相关。典型病例的影像学结合病理学通常可以诊断,但应结合免疫组化和其他组织学对相似的疾病进行鉴别。
Objective To study the clinicopathological and immunohistochemical feature of Hemangioblastoma.Methods Eighteen patients with pathologically confirmed hemangioblastoma were retroactively included into the research. Clinicopathology、immunohistochemistry and preoperative Imaging appearances on MRI system were obtained and compared among every patient.Results There were 18 cases of sporadic occurrence,16 cases of first episode and 2 cases of recurrence in the research. The first onset of age ranged 8 - 61 years old. The tumors were situated in the cerebellum with 15 cases,in the spinal cord with 2 cases and in the cerebellopontine angle with 1 case. Histopathologically,tumors were composed of different mature stages capillaries and stromal cells. The immunohistochemistry of Vim were positive expression in all the patients. In addition,positive expression of CD34 and EGFR were found in 12 cases and there were positive NSE in 10 cases and positive S-100 in 9 cases with low Ki-67 index. Conclusion Hemangioblastoma is more common in young and middle-aged with preferred sites of cerebellum and spinal cord,which is characterized by numerous capillary and stromal cells. But there are exception to the age and site of disease. The diagnostic performance of combined clinicopathology and Imaging appearances is superior to individual each. Further combination the both with immunohistochemistry improve the ability of differential diagnosis compared with other similar diseases.
作者
田海萍
黑静雅
马莉琼
孙金萍
TIAN Haiping ;HEI Jingya;MA Liqiong ;SUN Jingping(Department of Pathology, The Genaral Hospital of Ningxia Medical Univer- city, Yinchuan , 750004, China)
出处
《宁夏医学杂志》
CAS
2018年第10期918-920,共3页
Ningxia Medical Journal