原发心脏雪旺细胞瘤

目的 探讨原发心脏雪旺细胞瘤临床特征、诊断及外科治疗。方法 报告1例原发恶性心脏雪旺细胞瘤的诊断及手术经过，并收集到世界文献报道18例资料，对其临床表现、诊断及外科治疗进行讨论。结果 19例中，恶性17例，良性2例，其中施行手术切除11例，良性肿瘤术后效果好，恶性肿瘤远期生存率低。结论 原发心脏雪旺细胞瘤极其罕见，诊断依靠临床表现、心脏超声、核磁共振检查、组织形态及免疫组化特征。良性心脏雪旺细胞瘤切除彻底可获得良好效果，恶性心脏雪旺细胞瘤由于局部伸长、浸润和远处转移不能彻底切除，预后不良。心脏移植治疗早期原发心脏恶性雪旺细胞瘤可望得到较好效果。

Primary cardiac schwannoma-a case report and literature review

Objective: To report one case of primary cardiac schwannoma (PCS) with review of literature. Methods: One patient with primary malignant cardiac schwannoma (PMCS) was treated surgically in our hospital and relevant data of 18 cases were collected from international literature. Clinical features, diagnosis and surgical treatment of PCS were discussed. Results: 17 cases were PMCS and 2 cases were primary benign cardiac schwannoma (PBCS). Operation was performed in 11 cases. The locations of mass were both superior vena cava and right atrium in 2 cases, inferior vena cava and right atrium in 1, pulmonary vein and left atrium in 1, right atrium in 1, left atrium in 3, right ventricular outflow tract in 2, and intrapericardial in 1. The long-term results for resected PCS were excellent, but for PMCS were very poor. Conclusion: PCS is an extremely rare disease. The diagnosis dependents on clinical features, M-mode and two-dimensional echocardiography, magnetic resonance imaging, and histopathological and immunohistochemical findings. It is concluded that most PBCS can be resected completely with good results. PMCS can not be resected completely either because of the extent spread and invasion or frequent distant metastasis. The prognosis is dismal and early cardiac transplantation probably offers the only hope for patients with PMCS.