原发性心脏恶性肿瘤的诊断与外科治疗

Diagnosis and surgical treatment of primary malignant cardiac tumors

目的探讨原发性心脏恶性肿瘤的诊断、外科治疗及预后。方法和结果1999年1月至2012年5月在第二军医大学长海医院手术治疗的原发性心脏恶性肿瘤患者共13例，占同期手术治疗的心脏肿瘤患者的9．2％（13／141）。术前均经超声心动图明确诊断。胸部正中切口12例，左前外侧切口1例。完整切除8例，部分切除1例，开胸探查并取活检4例。肿瘤起源于右心房3例，右心室3例，左心房4例，左心室1例，同时累及右心房、右心室2例。术后病理结果：血管肉瘤3例，横纹肌肉瘤3例，间叶肉瘤3例，平滑肌肉瘤2例，未分化肉瘤1例，纤维黏液样肉瘤1例。无手术或院内死亡，术后均获得随访。术后中位生存时间17．5个月（1～76个月），肿瘤完整或大部分切除的患者术后中位生存时间18．5个月，仅取活检的患者术后中位生存时间8个月。结论原发性心脏恶性肿瘤多发于右心系统，心脏超声是最重要的诊断工具；心脏恶性肿瘤预后很差，手术切除是治疗心脏恶性肿瘤、改善预后的良好方法。

Objective To discuss the diagnosis, surgical treatment, and prognosis of primary malignant cardiac tumors. Methods and Results The clinical data of 13 patients with primary malignant cardiac tumors, who were surgically treated from January,1999 to May, 2012, were retrospectively analyzed. The 13 patients accounted for 9.22% （13/141） of patients with cardiac tumors who were surgically treated during the corresponding period. The diagnoses of the 13 cases were all comfirmed by echocardiography. Median sternotomy was performed in 12 cases and left anterolateral thoracotomy in 1. Complete resection of tumor was performed in 8 cases, subtotal resection in 1, and only biopsy in 4. The tumors arose from the right atrium in 3 cases, right ventricle in 3, left atrium in 4, left ventricle in 1, and right atrium combined with right ventricle in 2 cases. Pathological diagnoses included angiosarcoma in 3 cases, rhabdomyosarcoma in 3, mesenchymal sarcoma in 3, leiomyosarcoma in 2, undifferentiated sarcoma in 1, and fibromyxoid sarcoma in 1. There was no operative or in-hospital death, and all the patients were followed up after surgery. The median survival time of the entire group was 17.5 months （range, 1-76 months）. The median survival time of the patients who underwent a complete or subtotal surgical excision was 18. 5 months compared with 8 months for those who underwent only biopsy. Conclusion Malignant primary cardiac tumors are often found in the right heart. Echocardiography is the most important diagnostic method. The prognosis of malignant primary cardiac tumors is very poor. Surgical excision remains an effective method to improve the prognosis.