本文選題：腎臟腫瘤　切入點：滑膜肉瘤　出處：《臨床與實驗病理學雜志》2017年07期

【摘要】：目的探討罕見的腎臟橫紋肌樣滑膜肉瘤的臨床病理學特征。方法回顧性分析1例腎臟橫紋肌樣滑膜肉瘤患者的臨床資料和病理學形態(tài),對其原發(fā)灶和轉移灶行免疫組化EnVision兩步法檢查及SS18-SSX融合基因檢測,并復習相關文獻。結果患者男性,31歲,2014年11月影像學檢查發(fā)現(xiàn)右腎腫瘤,行右腎根治性手術,鏡下見腫瘤細胞呈短梭形,彌漫排列,胞質豐富,可見嗜伊紅團塊樣物,初次病理診斷為腎臟橫紋肌樣瘤。2015年10月影像學檢查發(fā)現(xiàn)肝臟及膈肌間占位,行肝臟腫瘤及部分膈肌切除,鏡下見腫瘤細胞均呈長梭形,細胞密集,交叉束狀排列,細胞質較少,胞質內未出現(xiàn)嗜伊紅團塊樣物,該形態(tài)學為典型滑膜肉瘤。免疫表型:EMA、TLE1、vimentin、CD56均陽性,INI1陰性。FISH檢測SS18-SSX融合基因,原發(fā)灶和轉移灶均為陽性,最終證實原發(fā)灶即為滑膜肉瘤。結論腎臟橫紋肌樣滑膜肉瘤罕見,易誤診為腎臟橫紋肌樣瘤,該例腎臟橫紋肌樣滑膜肉瘤拓寬了腎臟橫紋肌樣腫瘤的鑒別診斷瘤譜,即使為典型的橫紋肌樣形態(tài),也需利用分子生物學手段進行鑒別診斷。SS18-SSX融合基因檢測陽性是確診滑膜肉瘤的依據(jù)。
[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.
【作者單位】： 復旦大學附屬中山醫(yī)院病理科;
【基金】：上海市衛(wèi)計委薄弱科室項目(2015ZB0201) 復旦大學附屬中山醫(yī)院青年基金(2015ZSQN17)
【分類號】：R737.11

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