Cardiology Department, Santa Marta Hospital, Central Lisbon Hospital University Center, Lisbon, Portugal.
Pathology Department, Santa Marta Hospital, Central Lisbon Hospital University Center, Lisbon, Portugal.
Oncology. 2023;101(5):292-302. doi: 10.1159/000528915. Epub 2023 Jan 19.
Malignant primary cardiac tumors are exceedingly rare, and despite surgical exeresis or chemotherapy, their prognosis remains poor. Cardiac invasion by metastatic tumors, while more common, also entails an unsatisfactory outcome. This study aimed to review patients diagnosed with malignant primary and secondary cardiac tumors in a tertiary center between 1995 and 2022.
Clinical data, echocardiographic, computed tomography, and magnetic resonance assessments of tumor location and morphology, histology, treatment, and survival were retrospectively analyzed.
Sixty malignant cardiac tumors were diagnosed: 17 primary (A) and 43 metastatic (B) tumors. A: the most common types were angiosarcoma (41%), undifferentiated sarcoma (23%), and fibrosarcoma (18%). Patients with primary tumors were younger than patients with metastatic tumors (41 ± 13 years vs. 57 ± 18 years, p = 0.001), with no significant gender difference. The most frequent presentations were heart failure (59%) and arrhythmia (23%). The most prevalent tumor location was the right heart chambers (71%), mostly in the right atrium (35%). 47% were submitted to tumor resection, and 29% received chemotherapy. The mortality rate was 82% with a median survival of 6.0 (interquartile range: 1.0-11.8) months after diagnosis (minimum of 12 days and maximum of 19 years). One patient with fibrosarcoma underwent heart transplantation and was still alive and well after 19 years. B: regarding metastatic cardiac invasion, the most common primary tumor sites were lung carcinomas (38%), thymomas (17%), and lymphomas (14%). Presentation with pericardial effusion was common (33%). The mortality rate was 72%, with a median survival of 3.6 (1.0-13.4) months (minimum of 7 days, maximum of 5 years).
Diagnosis of metastatic cardiac tumors was more common than that of malignant primary tumors, both with a dismal prognosis. When radical exeresis is not possible, heart transplantation can be an option with a favorable outcome in carefully selected patients with sarcomas.
恶性原发性心脏肿瘤极为罕见，尽管进行了手术切除或化疗，但其预后仍然较差。转移性肿瘤侵犯心脏虽然更为常见，但结果也并不理想。本研究旨在回顾 1995 年至 2022 年期间在一家三级中心诊断为恶性原发性和继发性心脏肿瘤的患者。
回顾性分析了肿瘤位置和形态、组织学、治疗和生存的临床数据、超声心动图、计算机断层扫描和磁共振评估。
共诊断出 60 例恶性心脏肿瘤：17 例原发性（A）和 43 例转移性（B）肿瘤。A 组中最常见的类型是血管肉瘤（41%）、未分化肉瘤（23%）和纤维肉瘤（18%）。原发性肿瘤患者比转移性肿瘤患者年轻（41 ± 13 岁 vs. 57 ± 18 岁，p = 0.001），且无明显性别差异。最常见的表现是心力衰竭（59%）和心律失常（23%）。最常见的肿瘤位置是右心腔（71%），主要在右心房（35%）。47%的患者接受了肿瘤切除术，29%的患者接受了化疗。诊断后死亡率为 82%，中位生存期为 6.0（四分位距：1.0-11.8）个月（最短 12 天，最长 19 年）。1 例纤维肉瘤患者接受了心脏移植，19 年后仍存活且状况良好。B 组中，转移性心脏侵犯最常见的原发肿瘤部位是肺癌（38%）、胸腺瘤（17%）和淋巴瘤（14%）。心包积液是常见的表现（33%）。死亡率为 72%，中位生存期为 3.6（1.0-13.4）个月（最短 7 天，最长 5 年）。
转移性心脏肿瘤的诊断比恶性原发性肿瘤更为常见，两者预后均较差。当无法进行根治性切除时，心脏移植可以作为一种选择，在精心挑选的肉瘤患者中可获得良好的结果。
