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目的探讨心脏滑膜肉瘤的临床病理学特征及鉴别诊断,提高对该肿瘤的认识。方法收集中山医院病理科2000~2012年心外科切除的37例心脏恶性肿瘤,复习患者的临床病理资料并进行随访,制备高产组织芯片,行免疫组化EnV ision两步法染色和SS18-SSX融合基因FISH检测。结果 37例心脏恶性肿瘤中7例为滑膜肉瘤,均为单相纤维型,6例表达EMA,7例SS18-SSX融合基因均阳性。男性5例,女性2例,发病年龄16~63岁。7例滑膜肉瘤均为手术切除标本,肿瘤原发于心包4例、右心房1例、同时累及右心房和右心室1例及左右心室交界处1例。7例患者均死亡,存活时间12~42个月。结论滑膜肉瘤是一种少见的软组织恶性肿瘤,尤其在发生于罕见部位心脏时,易漏检,诊断具有一定的挑战性。诊断根据其组织形态学,免疫组化染色与SS18-SSX融合基因检测可协助诊断。
Objective To investigate the clinicopathological features and differential diagnosis of cardiac synovial sarcoma and to raise awareness of the tumor. Methods Thirty-seven patients with cardiac cancer resected from 2000 to 2012 in Department of Pathology, Zhongshan Hospital were enrolled in this study. The clinical and pathological data were reviewed and followed up. High-yielding tissue microarray was prepared. Two-step EnV ision immunohistochemistry and SS18-SSX fusion Gene FISH test. Results Seven of 37 cardiac malignant tumors were synovial sarcomas, all of which were single-phase fibrosis. EMA was expressed in 6 cases and positive in 7 cases of SS18-SSX fusion gene. 5 males and 2 females, the age of onset of 16 to 63 years old. Seven cases of synovial sarcomas were resected specimens, the tumor originated in the pericardium in 4 cases, 1 case of right atrium, involving both the right atrium and right ventricle in 1 case and left and right ventricular junction in 1 case. All 7 patients died, with a survival time of 12-42 months. Conclusions Synovial sarcoma is a rare malignant tumor of soft tissue. Especially when it occurs in the heart of a rare site, it is easy to undetect and diagnose it. Diagnosis According to their histomorphology, immunohistochemistry and SS18-SSX fusion gene test can help diagnose.