原发性心脏肉瘤8例临床病理分析

目的:分析原发性心脏肉瘤的临床病理学特征及其预后与分级之间的关系。方法:回顾性分析8例原发性心脏肉瘤的临床资料、组织学形态及免疫组化结果，并进行随访。所有肉瘤均按照FNCLCC分级系统进行分级。结果:心脏肉瘤8例，年龄30~60岁（平均年龄45岁），男女比例为1∶3，临床表现无特异性，发生部位:左心房4例，左心室1例，右心房1例，右房、右室及部分右室流出道1例，双侧心房1例。肿瘤直径从17 mm到93 mm。具体组织学类型:未分化多形性肉瘤3例，血管肉瘤、上皮样血管内皮瘤、平滑肌肉瘤、胚胎性横纹肌肉瘤、去分化脂肪肉瘤各1例。所有肿瘤被分级为1级（2例）、2级（1例）、3级（5例）。除2例失访外，其余6例均获得随访结果，随访时间4~84个月(中位时间40个月），其中2例低级别（1级）肿瘤患者均未见复发及转移，4例中、高级别（2、3级）肿瘤患者在术后4~30个月死亡。结论:心脏肉瘤罕见，其中未分化多形性肉瘤与血管肉瘤多见，患者的总体预后差，与肿瘤的分级有关，由于本组样本量少，无法做统计学分析，需要更大的样本量来判断两者有无统计学相关性。

Report and clinicopathologic study of 8 cases of primary cardiac sarcomas

Objective:To analyze the clinicopathological features of primary cardiac sarcomas and the relationship between prognosis and the grade.Methods:A retrospective study was performed in 8 cases of primary cardiac sarcomas.All sarcomas were graded according to the FNCLCC system.The follow-up results were obtained by telephone.Results:There were 8 cases of cardiac sarcomas.Age ranged from 30 to 60 years (mean 45 years),male∶female =1∶3.Their clinical manifestations are nonspecific.The tumors involved the left atrium in 4 cases,left ventricle in1 case,right atrium in 1case,right ventricle,right atrium and partial right ventricle outflow tract in 1case,bilateral atrium in 1 case.The tumor size ranged from 17 mm to 93 mm.Histologically,there were 3 undifferentiated pleomorphic sarcomas,1 angiosarcoma,1 epithelioid hemangioendothelioma,1 leiomyosarcoma,1 embryonal rhabdomyosarcoma,and 1 dedifferentiated liposarcoma.All tumors were graded as 1 (2 cases),2 (1 cases),and 3 (5 cases).6 cases were followed up,follow-up time ranged from 4~84 months(median 40 months).2 low grade (grade 1) cases had no tumor recurrence and metastasis,4 middle and high grade(2,3) cases died of disease after operation ranged from 4 to 30 months.Conclusion:Primary cardiac sarcoma is rare.Undifferentiated pleomorphic sarcoma and angiosarcoma are the most common types.The overall outcome of the patients were poor,which related with tumor grade.Because of only a few of cases in this series,we cannot do statistical analysis.Larger series are needed to analyze to find whether there were statistically significant or not.