上皮样肉瘤的临床病理及免疫组织化学分析

Clinicopathologicobservation and Immunohistochemical Study of Epithelioid Sarcoma

目的探讨上皮样肉瘤(ES)的临床病理学特征、诊断和鉴别诊断。方法收集13例ES,其中近端型9例,经典型4例。研究分析其临床资料、病理形态学及免疫组织化学分析结果,分析INI-1、CyclinD1二者表达的相关性。结果近端型以上皮样细胞为主,呈多结节状及弥漫片状生长方式;经典型兼具有梭形细胞及上皮样细胞,似肉芽肿样结构。免疫组织化学分析显示,肿瘤细胞Vimentin、CKpan、CD34、EMA、CyclinD1阳性,Desmin、S-100为弱阳性,CD31、CA125、HMB45、MelanA均为阴性。INI-l在经典型和近端型中的失表达率分别为77.8%(7/9)和75.0%(3/4),二者比较差别无统计学意义(P>0.05)。INI-1与CyclinD1蛋白表达无明显相关性(P>0.05)。结论 ES少见,易误诊,结合病史,联合应用INI-1、Vimentin、CKpan、EMA、CD34等指标对ES的诊断及鉴别诊断有帮助;在ES中存在INI-1失表达与CyclinD1过表达现象,但二者无明显相关性。

Objective To explore the clinicopathologic features,diagnosis and differential diagnosis of epithelioid sarcoma（ES）. Methods The clinical data,histopathologic observation and immunohistochemical staining were studied in 13 cases of ES. Correlation of INI-l and CyclinD1 protein expression was analyzed. Results Among the 13 cases of ES,4were conventional ES and 9were of proximal-type ES. Proximal-type ES consisted of epithelioid cells,which showed an infiltrative growth pattern with nodular and sheet arrangement. Conventional ES consisted of epithelioid cells and spindle cells,with a granuloma-like pattern. The immunohistochemical study showed expression of Vimentin,CKpan,CD34,EMA,CyclinD1. The immunostain for Desmin,S100,which was focal and weak was found.CD31,CA125,HMB45 and MelanA were negative in all cases. The loss of INI-1was found in 77.8%（7/9）and 75.0%（3/4）in the conventional ES and the proximal-type ES,respectively. There were no significant differences in negative rate of INI-1protein between the conventional ES and the proximal-type ES（P〉0.05）. The loss expression of INI-1protein is not associated with the high expression of CyclinD1protein（P〉0.05）. Conclusions Owing to low incidence,pitfalls in diagnosis of ES sometimes are encountered. Immunostaining for INI-1combined with other commonly used markers（Vimentin,CKpan,EMA and CD34）will be helpful for correct diagnosis. Upregulation of CyclinD1 expression and loss of INI-1expression was found in ES and there is not association between the two.