原发性心滑膜肉瘤4例临床病理分析  

Primary cardiac synovial sarcoma:a clinicopathologic analysis of four cases

作　　者：霍真[1] 赵雨[1] 杨堤[1] 李媛[1] 罗玉凤[1] 曹金伶[1] 梁智勇[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院病理科,北京100730

出　　处：《诊断病理学杂志》2016年第1期15-18,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的报道4例原发性心滑膜肉瘤，并总结分析此类疾病的临床病理特征。方法通过光镜、免疫组化及荧光原位杂交技术（FISH）分析其临床病理特征及免疫组化特点。结果本组4例心滑膜肉瘤年龄22～52岁，平均41岁；男女之比为1：3，均无滑膜肉瘤病史。1例有吸烟史。4例肿瘤最大径6～24cm，平均12．5cm；2例肿瘤位于心包，1例位于心房，1例位于心室。均行肿瘤局部切除术。镜下检查3例为单相型滑膜肉瘤，1例为双相型滑膜肉瘤。4例随访1天～24个月，3例死亡，1例存活。免疫组化：4例AEl／AE3、EMA、vimentin和bcl-2均（＋），3例CD99（＋），3例calponin（＋），1例S-100散在（＋）；4例desmin、SMA、CD34和calretinin均（-）；Ki一67阳性指数5％～35％，平均18％。结论原发性心滑膜肉瘤比较少见，诊断应首先排除转移性。单相型滑膜肉瘤较双相型多见，心包为最常见部位。原发性心滑膜肉瘤预后较差，特别是心包滑膜肉瘤预后极差，术后放、化疗有助于延长生存时间。Objective To investigate the clinical features, pathological and differential diagnosis of primary cardiac synovial sarcoma（PCSS） over a period of 15 years from 2000 to 2014. Methods All the clinical and histological findings, immunophenotype, treatment and prognosis in all 4 cases of PCSS were evaluated with review of the relevant literature. Results 4 cases of PCSS included 3 females and 1 male, aged 22 -52 years （mean, 41 years）, 1 case had a history of smoking. 4 cases had no history of synovial sarcoma. The diameters of the tumors ranged from 6 - 24 cm （mean, 12. 5 cm）. 2 cases located in the pericardium, and 1 case located in the atrium, and 1 case located in the ventricle. 4 cases underwent partial tumor resection. Microscopically, 3 cases were single-phase synovial sarcoma, 1 case was biphase synovial sarcoma. Follow-up data were available in 4 cases from one day-24 months） ： 3 cases died, and 1 case was alive. Immunohistochemically, AE1/AE3, EMA, vimentin and bcl-2 were positive in all 4 cases; CD99 and calponin were positive in 3 cases; S-100 was focal positive in 1 case; Desmin, SMA, CD34 and calretinin were negative; Ki-67 index ranged from 5% to 35% （ mean, 18% ）. Conclusion Primary cardiac synovial sarcoma is rare, and metastatic tumor should be ruled out in diagnosis. The number of single-phase synovial sarcoma is more than biphase synovial sarcoma, and pericardium is the most common site. Prognosis is poor, especially the synovial sarcoma of the pericardium, and postoperative chemotherapy helps to prolong the survival time.

关 键 词：心 滑膜肉瘤 免疫组化 

分 类 号：R731.1[医药卫生—肿瘤]