肾脏横纹肌样滑膜肉瘤1例并文献复习

发布时间：2018-04-01 11:38

本文选题：肾脏肿瘤　切入点：滑膜肉瘤　出处：《临床与实验病理学杂志》2017年07期

【摘要】：目的探讨罕见的肾脏横纹肌样滑膜肉瘤的临床病理学特征。方法回顾性分析1例肾脏横纹肌样滑膜肉瘤患者的临床资料和病理学形态,对其原发灶和转移灶行免疫组化EnVision两步法检查及SS18-SSX融合基因检测,并复习相关文献。结果患者男性,31岁,2014年11月影像学检查发现右肾肿瘤,行右肾根治性手术,镜下见肿瘤细胞呈短梭形,弥漫排列,胞质丰富,可见嗜伊红团块样物,初次病理诊断为肾脏横纹肌样瘤。2015年10月影像学检查发现肝脏及膈肌间占位,行肝脏肿瘤及部分膈肌切除,镜下见肿瘤细胞均呈长梭形,细胞密集,交叉束状排列,细胞质较少,胞质内未出现嗜伊红团块样物,该形态学为典型滑膜肉瘤。免疫表型:EMA、TLE1、vimentin、CD56均阳性,INI1阴性。FISH检测SS18-SSX融合基因,原发灶和转移灶均为阳性,最终证实原发灶即为滑膜肉瘤。结论肾脏横纹肌样滑膜肉瘤罕见,易误诊为肾脏横纹肌样瘤,该例肾脏横纹肌样滑膜肉瘤拓宽了肾脏横纹肌样肿瘤的鉴别诊断瘤谱,即使为典型的横纹肌样形态,也需利用分子生物学手段进行鉴别诊断。SS18-SSX融合基因检测阳性是确诊滑膜肉瘤的依据。
[Abstract]:Objective to investigate the clinicopathological features of rare renal rhabdomyoid synovial sarcomas. Methods A case of renal rhabdomyoid synovial sarcoma was retrospectively analyzed. The primary and metastatic tumors were examined by immunohistochemical EnVision two-step method and SS18-SSX fusion gene detection, and the related literatures were reviewed. Results the male patients were 31 years old. In November 2014, the tumor of the right kidney was found by imaging examination, and the radical operation of the right kidney was performed. Under the microscope, the tumor cells showed short fusiform, diffuse arrangement, abundant cytoplasm, eosinophilic mass, and primary pathological diagnosis of renal rhabdomyoma. In October 2015, the liver and diaphragm were found to occupy the space between the liver and the diaphragm. Liver tumors and partial diaphragmatic resection were performed. Under microscope, tumor cells were shown to be fusiform, dense cells, cross bundles, few cytoplasm, and no eosinophilic mass in cytoplasm. The morphology was typical synovial sarcomas. Immunophenotype: EMA TLE1 vimentinCD56 was positive and INI1 negative. Fish was positive for SS18-SSX fusion gene. The primary focus and metastasis were both positive, and the primary tumor was proved to be synovial sarcoma. Conclusion rhabdomyoid synovial sarcoma of kidney is rare. This case of renal rhabdomyoid synovial sarcoma broadens the differential diagnosis of renal rhabdomyoid tumor, even if it is a typical rhabdomyoid tumor. Molecular biological methods should also be used for differential diagnosis. SS18-SSX fusion gene positive is the basis for the diagnosis of synovial sarcoma.
【作者单位】：复旦大学附属中山医院病理科;
【基金】：上海市卫计委薄弱科室项目(2015ZB0201) 复旦大学附属中山医院青年基金(2015ZSQN17)
【分类号】：R737.11

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