儿童横纹肌肉瘤的上皮和神经内分泌标志物

Epithelial and neuroendocrine markers in rhabdomyosarcoma in children

目的:探讨上皮和神经内分泌标志物细胞角蛋白(cytokeratin,CK)、突触素(synaptophysin,Syn)和嗜铬粒蛋白A(chromogranin A,CgA)在儿童横纹肌肉瘤(rhabdomyosarcoma,RMS)中的表达及临床病理意义。方法:回顾性分析56例RMS的临床特征、组织学形态,应用免疫组织化学的方法检测CK,Syn,CgA在各型RMS组织中的表达,结合随访结果进行分析。结果:52例患儿中,男38例,女18例,年龄24 d^9岁(平均3岁11个月)。发生部位:以泌尿生殖道最为常见(16/56,28.6%)。镜下:56例中,诊断胚胎性RMS(epithelioid rhabdomyosarcoma,ERMS)32例、腺泡状RMS(alveolar rhabdomyosarcoma,ARMS)19例、梭形细胞RMS(spindle cell rhabdomyosarcoma,SCRMS)5例。免疫表型:41.1%(23/56)表达广谱CK,43.1%(22/51)表达Syn,22.4%(11/49)表达CgA;其中,Syn和CgA的表达方式呈局灶性,并且有22%的病例同时表达二者;9%的病例同时表达CK和Syn或CgA。结论:各种类型的RMS中均可不同程度地表达上皮和神经内分泌标志物,提示可使用这一组标志物诊断和鉴别诊断RMS与神经内分泌肿瘤及其他小圆形细胞肿瘤。

Objective: To evaluate the expression and clinicopathological significance of epithelial and neuroendocrine markers: cytokeratin(CK), synaptophysin(Syn), chromogranin A(CgA) in rhabdomyosarcoma of children. Methods: A retrospective analysis of 56 cases of rhabdomyosarcoma was made. Immunohistochemical method was used to detect the expression levels of CK, Syn and CgA in rhabdomyosarcoma tissues of various types, and the follow-up results was analyzed. Results: Th ere were 38 males and 18 females with an average age of 3 years and 11 months. Location of occurrence: urogenital tract was the most common(16/56, 28.6%). Microscopically, 32 cases of embryonic rhabdomyosarcoma, 19 cases of alveolar rhabdomyosarcoma and 5 cases of spindle cell rhabdomyosarcoma were diagnosed in 56 cases. Immunophenotype: 41.1%(23/56) of patients expressed CK, 43.1%(22 of 51) expressed Syn, and 22.4%(11/49) expressed CgA;the expression of syn and CgA was localized, and 22% expressed both. CK and Syn or CgA were expressed simultaneously in 9% of cases. Conclusion: Th ese epithelial and neuroendocrine markers can be expressed to varying degrees in various types of RMS. Th is finding highlights the need to employ a panel of markers to diagnose and differentiate rhabdomyosarcoma from neuroendocrine tumors and other small round cell tumors.