Coli Antonella, Chiariello Giovanni Alfonso, Novello Mariangela, Colizzi Christian, Massetti Massimo
Institute of Anatomic Pathology, Catholic University of Sacred Heart, Largo F. Vito 1, 00168, Rome, Italy.
Department of Cardiovascular Sciences, Catholic University of Sacred Heart, Rome, Italy.
J Cardiothorac Surg. 2018 Jul 3;13(1):84. doi: 10.1186/s13019-018-0771-0.
Primary heart sarcomas are exceedingly rare tumors. Among primary cardiac sarcomas, synovial sarcoma is one of the rarest, involving cardiac cavities or pericardium.
Two cases of synovial sarcoma are presented with the clinical course and therapy. Both cases were treated with surgery and chemo/radiotherapy. Interestingly, one of the patient, a 52-year-old male with an intracardiac synovial sarcoma, undergone a SynCardia total artificial heart implantation, but died for multiple pulmonary metastases waiting for transplantation.
Complete surgical resection of cardiac synovial sarcoma is the gold standard of therapy, though rarely possible. Although guidelines for the treatment are not well established, due to limited number of cases reported, chemotherapy and radiotherapy are frequently administered and seem to prolong mean patient's survival. Cardiac transplantation could be considered in selected cases.
原发性心脏肉瘤是极为罕见的肿瘤。在原发性心脏肉瘤中，滑膜肉瘤是最罕见的类型之一，可累及心腔或心包。
本文介绍了两例滑膜肉瘤的临床病程及治疗情况。两例均接受了手术及化疗/放疗。有趣的是，其中一名患者为52岁男性，患有心内滑膜肉瘤，接受了SynCardia全人工心脏植入术，但在等待移植期间因多发肺转移而死亡。
心脏滑膜肉瘤的完整手术切除是治疗的金标准，尽管很少能够实现。由于报道的病例数量有限，治疗指南尚未完善，但化疗和放疗经常应用，似乎可延长患者的平均生存期。在特定病例中可考虑心脏移植。
