神经系统髓外浆细胞瘤临床病理学观察  

Nervous System Extramedullary Plasmacytoma:A Clinicopathologic Study of 3 Cases

作　　者：何妙侠[1] 王建军[1] 姜秀峰[2] 蒋慧[1] 陶立阳[1] 

机构地区：[1]第二军医大学长海医院病理科,上海200433 [2]第二军医大学长海医院神经外科,上海200433

出　　处：《中国神经肿瘤杂志》2010年第1期26-30,共5页Chinese Journal of Neuro-Oncology

摘　　要：背景与目的:发生于神经系统的髓外浆细胞瘤罕见。本研究分析神经系统髓外浆细胞瘤的临床病理特点。方法:总结3例神经系统髓外浆细胞瘤临床病理特点,EnVision法分析免疫表型(LCA、CD20、CD79a、CD3、CD7、PC、MUM1、CD138、Ki-67),PCR法分析IgH和TCRγ基因重排,并进行治疗随访。结果:3例神经系统髓外浆细胞瘤,2例发生于大脑,均为男性,年龄为48和64岁。1例发生于T10椎管内,女性,29岁。影像学均提示占位性病变,临床表现为头痛呕吐,病理学特征为:不同分化程度的浆细胞样肿瘤细胞弥漫性浸润,间质内血管丰富或形成血湖,可见淀粉样物质沉积。免疫表型:3例之瘤细胞均表达CD79a,PC和MUM1,均不表达CD20和CD3、CD7。基因重排分析结果显示IgH基因均呈单克隆性重排。结论:神经系统髓外浆细胞瘤具有特征性的临床病理学改变,免疫组化和基因重排分析能够帮助确定肿瘤细胞起源,诊断时应综合考虑,并排除多发性骨髓瘤。BACKGROUND OBJECTIVE：Extramedullary Plasmacytoma is rare in nervous system.In this article,we investigated the clinicopathological features of nervous system extramedullary plasmacytoma.METHOD：Clinical,pathological features and follow-up of 3 cases of nervous system extramedullary plasmacytoma（EMP） were reviewed.Immunohistochemical staining was performed by EnVision method using antibodies LCA,CD19,CD20,CD79a,CD3,CD7,PC,MUM1,Ki-67.IgH and TCRγ gene rearrangement was detected by polymerase chain reactive.RESULT：Clinically,the neoplasms were located in nervous system,2 in male patients with the age of 48 years old and 64 years old respectively and 1 in T10 vertebral canal of a 29 years-old female.All patients were all had complains of headache and vomitting.Histopathological examination revealed diffusely invasive tumor cells with different degree differentiation.There were a few interstitial tissues with plenty of vessels or formation of bloodlakes.And there was deposition of amyloid materials.Immunophenotypic analysis showed that all tumor cells were positive for CD79a,PC and MUM1,and negative for CD20.PCR showed monoclone gene rearrangement of IgH gene.CONCLUSION：All the specific clinicopathological features in the nervous system extramedullary plasmacytoma is useful for diagnosis.The origin of tumor cells was confirmed by immunohistochemistry and gene rearrangement analysis.The multiple myeloma must be excluded.

关 键 词：髓外浆细胞瘤 神经系统 临床病理 免疫表型 基因重排 

分 类 号：R739.41[医药卫生—肿瘤]