眼内平滑肌瘤临床特点及病理学观察  

THE CLINICAL CHARACTERISTICS AND HISTOLOGY OF MESECTODERMAL LEIOMYOMA

作　　者：莫宾[1] 岳常丽[2] 刘红刚[2] 刘武[1] Mo Bin Yue Changli Liu Honggang et al(Beijing Tongren Eye Center ,Beijing Tongren Hospital, Capital Medi- cal University ,Beijing Ophthalmology ~ Visual Sciences key Laboratory ,Beijing 100730 ,China.)

机构地区：[1]首都医科大学附属北京同仁医院眼科中心北京市眼科学与视觉科学重点实验室,北京市100730 [2]首都医科大学附属北京同仁医院病理科头颈部分子病理诊断北京市重点实验室

出　　处：《中国煤炭工业医学杂志》2017年第7期795-800,共6页Chinese Journal of Coal Industry Medicine

摘　　要：目的探讨脉络膜平滑肌瘤的临床病理特点,提高其诊断与鉴别诊断水平。方法收集2004年1月—2014年12月北京同仁医院眼科眼内平滑肌瘤5例,行HE及免疫组织化学染色观察。结果患者平均年龄35.4岁,其中男1例,女4例,平均随访4.5年。眼底检查见周边或后极部无色素性脉络膜占位病变,合并有视网膜脱离。同时,所有患者行眼部超声检查,其中2例行了超声造影检查;所有患者均行增强MRI检查。超声检查显示球内占位性病变,边界清晰,可见或未见挖空征;2例行超声造影检查的患者,瘤体内均可见造影剂完全充填;增强MRI结果均显示眼内占位性病变并视网膜脱离,T1、T2加权像等信号影,增强MRI眼内实性占位,造影剂呈典型"快进-快出"型。组织学瘤细胞呈梭形、卵圆形;胞浆淡染细颗粒状;弥漫阳性表达vimentin、SMA,部分病理局灶阳性表达Syn、Chg-A、CD56或PR;阴性表达AE1/AE3、S-100、HMB-45和CD117。结论眼内平滑肌瘤少见,尤其是后极部脉络膜平滑肌瘤更为罕见。临床上与无色素脉络膜黑色素瘤、神经鞘瘤、髓上皮瘤等鉴别困难。详细的检查眼底、巩膜透照试验、眼部超声造影检查及增强MRI检查,并结合患者的一般情况及病史,综合对肿瘤性质进行判断。病理检查可明确诊断,瘤细胞伴有肌源性及神经源性免疫表达提示其可能来源于神经嵴。Objective To investigate the clinical and pathological features of choroidal leiomyoma and to improve the diagnosis and differential diagnosis of choroidal leiomyoma.Methods We reviewed 5 eyes of 5 patients with intraocular leiomyoma,who were diagnosed at author’s hospital from January 2004 to December2014.All patients underwent histopathological and immunohistochemistry examinations.Results The average age of the patients was 35.4 years old,including 1 male and 4 female,with an average follow-up of 4.5years.In the fundus examination,there was amelanotic choroidal mass in the surrounding or posterior pole,which was associated with retinal detachment.At the same time,all patients underwent eye ultrasonography,2 of whom underwent contrast-enhanced ultrasonography,and all patients underwent enhanced MRI scan.Ultrasound examination showed well-marginated,hemispherical mass accompanied by a large amount of subretinal fluid.Boundary,no visible or hollowed sign;ultrasound contrast examination was done in 2 cases which showed that the intraocular solid lesions were filled with contrast agents.The signal intensity of the mass was iso-intensity to contralateral vitreous on T1-weighted images and T2-weighted images.MRI enhancement showed that the intraocular solid lesions were filled with contrast agents that mainly showed as rapid increase,and the dissipation speed of the contrast agent was faster than that of normal tissues,indicating that the contrast agent has a short duration in the lesions,being a typical fast-in and fast-out pattern.Microscopically,the tumor composed mainly of ovoid or spindle-shaped with fibrillary cytoplasm,without evidence of mitotic activity in routine stains.Immunohistochemistry showed positivity for desmin,vimentin,smooth muscle actin（SMA）,progesterone receptor（PR）,synaptophysin（Syn）,Chromogranin-A（ChgA）and focal NCAM（CD56）.No reactivity with AE1/AE3,epithelial membrane antigen（EMA）,CD117,Estradiol Receptor（ER）,Androgen Receptor（AR）,S-100,HMB-45 or NSE was seen

关 键 词：平滑肌瘤 眼内 睫状体 脉络膜 病理 

分 类 号：R739.7[医药卫生—肿瘤]