神经内神经束膜瘤16例临床病理学分析  

Intraneural perineurioma: a clinicopathological analysis of 16 cases

作　　者：董荣芳 郑丹枫 孙晓淇 丁宜 DONG Rongfang;ZHENG Danfeng;SUN Xiaoqi;DING Yi(Department of Pathology,Beijing Jishuitan Hospital,Capital Medical University,Beijing 100032,China;Department of Pathology,Peking University Health Science Center/Peking University Third Hospital,Beijing 100089,China)

机构地区：[1]首都医科大学附属北京积水潭医院病理科,北京100032 [2]北京大学医学部病理系暨北京大学第三医院病理科,北京100089

出　　处：《临床与实验病理学杂志》2023年第10期1232-1235,1240,共5页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨神经内神经束膜瘤(intraneural perineurioma,InP)的临床病理学特征。方法回顾性分析16例InP的临床病理学及免疫表型特征,并复习相关文献。结果16例InP患者中男性5例,女性11例,年龄5~56岁,平均年龄27.4岁,14例为单发病变(分别位于尺神经、正中神经、桡神经、腓总神经),2例为多发病变(分别位于双侧臂丛神经及C1-3神经根)。患者术前病程2~360个月,常表现为病变神经对应区域缓慢进展的运动功能障碍,部分有感觉功能异常;影像学提示局部神经增粗呈纺锤形或长梭形,边界尚清,肌电图提示周围神经损伤;光镜下见梭形的神经束膜细胞以轴突-施万细胞复合体为中心形成假洋葱球样结构。电镜下见长而细的神经束膜细胞胞质内富含吞噬囊泡,与其伴随的施万细胞一起围绕轴突呈同心圆状排列。免疫表型:神经束膜细胞EMA、Glut-1、Claudin-1阳性,中心的轴突-施万细胞复合体NF、S-100及SOX10阳性。Ki-67增殖指数<5%。结论InP是一种罕见的起源于神经束膜的良性肿瘤,易与肥大性周围神经病、腓骨肌萎缩症、混杂性神经鞘膜肿瘤、神经鞘瘤、神经纤维瘤等混淆,熟练掌握其特征性的组织学表现及免疫表型是诊断关键。Purpose To investigate the clinicopathological characteristics of intraneural perineurioma(InP).Methods The clinical morphological and immunohistochemical features of 16 cases of InP were retrospectively analyzed,and the literatures were also reviewed.Results There were 5 females and 11 males,aged from 5-56 years(mean 27.4 years).14 cases were located in major peripheral nerves and their branches and 2 cases were multiple lesions.The duration of symptoms ranged 2 months to 360 months before excision.They presented as a slowly progressive painless mass with a loss of motor and less sensory function.MRI showed fusiform expansion of peripheral nerve.Histopathology typically showed characteristic pseudo-onion bulbs composed of whorls of spindle-shaped cells surrounding a center of residual axon-Schwann cell complex.Immunohistochemistry showed the perineural cells were positive for EMA,Glut-1,Claudin-1 and negative for NF,S-100,SOX10.Ki-67 proliferation index was all less than 5%.Conclusion InP is a rare benign perineural nerve tumor,prone to misinterpretation as peripheral nerve shealth tumor and synovial sarcoma,and immunohistochemical stains are helpful for diagnosis.Most cases have a good prognosis,and local recurrence may be associated with subtotal resections.

关 键 词：神经束膜瘤 神经内神经束膜瘤 假洋葱球样结构 周围神经 

分 类 号：R739.43[医药卫生—肿瘤]