Synovial sarcoma is a high-grade soft tissue malignancy characterized by a unique fusion gene known as SS18-SSX.The SS18-SSX fusion protein acts as an oncogenic driver of synovial sarcoma,and it has thus been commonly...Synovial sarcoma is a high-grade soft tissue malignancy characterized by a unique fusion gene known as SS18-SSX.The SS18-SSX fusion protein acts as an oncogenic driver of synovial sarcoma,and it has thus been commonly accepted that disruption of SS18-SSX function represents a therapeutic means of treating synovial sarcoma,but emerging evidence suggests that upon depletion of SS18-SSX,an anti-apoptotic signal surprisingly arises to protect synovial sarcoma cell survival.In this article,we discuss the controversial roles of SS18-SSX’s transcriptional activity in synovial sarcoma biology and outline a synergistic strategy for overcoming the resistance of synovial sarcoma cells to SS18-SSX targeted therapeutics.展开更多
文摘Synovial sarcoma is a high-grade soft tissue malignancy characterized by a unique fusion gene known as SS18-SSX.The SS18-SSX fusion protein acts as an oncogenic driver of synovial sarcoma,and it has thus been commonly accepted that disruption of SS18-SSX function represents a therapeutic means of treating synovial sarcoma,but emerging evidence suggests that upon depletion of SS18-SSX,an anti-apoptotic signal surprisingly arises to protect synovial sarcoma cell survival.In this article,we discuss the controversial roles of SS18-SSX’s transcriptional activity in synovial sarcoma biology and outline a synergistic strategy for overcoming the resistance of synovial sarcoma cells to SS18-SSX targeted therapeutics.
文摘目的：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EnVision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。
