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摘要: 滑膜肉瘤是一类具有间叶和上皮双向分化的恶性肿瘤，可发生于任何部位，好发于四肢深部软组织，而原发心脏的滑膜肉瘤非常罕见。临床主要表现为心腔阻塞、栓塞和填塞而引起的相应症状。当发生部位不典型时，诊断往往具有挑战性，确诊依靠组织病理学形态、免疫组织化学及分子遗传学检查(SYT-SSX融合基因改变)。我们对1例原发心脏滑膜肉瘤的临床资料、病理特征、治疗及随访进行分析，并复习相关文献，以提高临床医师及病理医师对该病的认识。
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关键词:
- 心脏滑膜肉瘤 /
- 临床病理特征 /
- SS18(SYT)-SSX融合基因 /
- 治疗 /
- 预后
Abstract: Synovial sarcoma is a type of malignant tumor with bidirectional differentiation of mesenchymal and epithelial cells, which can occur in any location. It mainly affects the deep soft tissues of the limbs, while primary synovial sarcoma of the heart is very rare. The main clinical manifestations are corresponding symptoms caused by cardiac obstruction, embolism, and tamponade. When the site of occurrence is atypical, diagnosis is often challenging, relying on histopathological morphology, immunohistochemistry, and molecular genetic examination(SYT-SSX fusion gene changes) for diagnosis. We analyzed the clinical data, pathological features, treatment, and follow-up of a case of primary cardiac synovial sarcoma, and reviewed relevant literature to enhance the understanding of the disease among clinical and pathological physicians. -
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表 1 基于免疫组化的心脏滑膜肉瘤的鉴别诊断
Table 1. Differential diagnosis of cardiac synovial sarcoma
肿瘤类型 CK EMA Vimentin SMA Desmin MyDO1 ERG CD34 STAT6 S100 Calretinin WT-1 SS +/- +/- + +/- +/- - - - - +/- +/- +/- IS - - + +/- +/- +/- +/- +/- - - - - Angiosarcoma +/- - + - - - + + - - - - MSFT - - + - - - - + + - - - MPNST - - + - +/- +/- - +/- - +/- - - MM + + + - - - - - - - + + Leiomyosarcoma +/- +/- + + + - - - - - - - RMS - - + + + + - - - - - - UPS - +/- + +/- - - - - - - - - 
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