Primary cardiac angiosarcoma is extremely rare, has a poor prognosis and has no specific clinical manifestation. A 31-year-old pregnant woman presented to the emergency department with signs of pericardial tamponade. Transesophageal echocardiography (TEE) showed a mass within her right atrium. Extensive surgical resection was performed with subsequent pericardial patch reconstruction of the right atrium. Histological examination showed the presence of an angiosarcoma. A reoperation was performed 18 months after the initial operation, because of concerns of radicality. Three years later she was re-admitted with a superior caval vein syndrome. A stent was placed to relieve symptoms. Because of the suspicion of a recurrent tumor, a second reoperation followed. During this operation, a perigraft hygroma was found pressing against the previously reconstructed superior caval vein. Histological examination after both reoperations showed no signs of a recurrent angiosarcoma. Until today, 8 years after her first surgery, the patient is still alive without any signs of recurrent tumor.

原发性心脏血管肉瘤极为罕见，预后差，无特异性临床表现。一名 31 岁的孕妇因心包填塞体征到急诊科就诊。经食道超声心动图 (TEE) 显示她的右心房内有肿块。进行了广泛的手术切除，随后对右心房进行了心包补片重建。组织学检查显示存在血管肉瘤。出于对激进性的担忧，在初次手术后 18 个月进行了再次手术。三年后，她因上腔静脉综合征再次入院。放置支架以缓解症状。由于怀疑肿瘤复发，随后进行了第二次再次手术。在此操作过程中，在先前重建的上腔静脉上发现了一个移植物周围的水瘤。两次再次手术后的组织学检查均未显示复发性血管肉瘤的迹象。直到今天，在她第一次手术 8 年后，患者仍然活着，没有任何肿瘤复发的迹象。