From Onco-Cardiology Program and Advanced Heart Failure Center (G.H.O., S.G.A.) and Department of Cardiac Surgery (S.J.P.), Harrington Heart and Vascular Institute, University Hospitals Case Medical Center, Cleveland, OH; and Seidman Cancer Center and Case Western Reserve University, Cleveland, OH (G.H.O., C.H.).
Circulation. 2015 Dec 22;132(25):2395-402. doi: 10.1161/CIRCULATIONAHA.115.016418. Epub 2015 Oct 14.
The aim of this study was to investigate the incidence, histopathology, demographics, and survival associated with primary malignant cardiac tumors (PMCTs).
We queried the Surveillance, Epidemiology and End Results (SEER) 18 registry from the National Cancer Institute for all PMCTs diagnosed from 1973 to 2011. We describe PMCT histopathology and incidence, comparing characteristics and survival of these patients with those of patients with extracardiac malignancies of similar histopathology. From a total of 7 384 580 cases of cancer registered in SEER, we identified 551 PMCTs (0.008%). The incidence of PMCT diagnosis is 34 cases per 100 million persons and has increased over time (25.1 in 1973-1989, 30.2 in 1990-1999, and 46.6 in 2000-2011). Most patients are female (54.1%) and white (78.6%) with median age at diagnosis of 50 years. The most common PMCTs are sarcomas (n=357, 64.8%), followed by lymphomas (n=150, 27%) and mesotheliomas (n=44, 8%). Most patients are diagnosed with tissue sample (96.8%). Although use of chemotherapy is not documented in SEER, 19% of patients received radiation and 44% had surgery. After a median follow-up of 80 months, 413 patients had died. The 1-, 3-, and 5-year survival rates were 46%, 22%, and 17% and have improved over the eras, with 1-, 3-, and 5-year survival rates of 32%, 17%, and 14% for 1973 to 1989 and 50%, 24%, and 19% for 2000 to 2011 (P=0.009). Cardiac sarcomas and mesotheliomas are the most lethal PMCTs, with 1-, 3-, 5-year survival rates of 47%, 16%, and 11% and of 51%, 26%, and 23% compared with 59%, 41%, and 34% for lymphomas, respectively (log rank test P<0.001). Patients with cardiac lymphomas and sarcomas are younger and have worse survival than patients with extracardiac disease of similar histopathology (P<0.001).
PMCTs are extremely rare and continue to be associated with poor prognosis. Over the past 5 decades, the incidence and survival of patients diagnosed with PMCT appear to have increased. Compared with those with extracardiac cancers of similar histopathology, patients with PMCTs are often younger and have worse survival.
本研究旨在探讨原发性心脏恶性肿瘤（PMCT）的发病率、组织病理学、人口统计学特征和生存情况。
我们从美国国立癌症研究所的监测、流行病学和最终结果（SEER）18 数据库中查询了 1973 年至 2011 年间诊断的所有 PMCT。我们描述了 PMCT 的组织病理学和发病率，并比较了这些患者与具有相似组织病理学的心脏外恶性肿瘤患者的特征和生存情况。在 SEER 登记的 7384580 例癌症病例中，我们共发现了 551 例 PMCT（0.008%）。PMCT 的诊断发病率为每 100 万人中有 34 例，且呈上升趋势（1973-1989 年为 25.1，1990-1999 年为 30.2，2000-2011 年为 46.6）。大多数患者为女性（54.1%）和白人（78.6%），中位诊断年龄为 50 岁。最常见的 PMCT 是肉瘤（n=357，64.8%），其次是淋巴瘤（n=150，27%）和间皮瘤（n=44，8%）。大多数患者通过组织样本诊断（96.8%）。尽管 SEER 未记录化疗的使用情况，但 19%的患者接受了放疗，44%的患者接受了手术。中位随访 80 个月后，413 例患者死亡。1、3 和 5 年生存率分别为 46%、22%和 17%，且随着时间的推移有所改善，1973-1989 年的 1、3 和 5 年生存率分别为 32%、17%和 14%，2000-2011 年的 1、3 和 5 年生存率分别为 50%、24%和 19%（P=0.009）。心脏肉瘤和间皮瘤是最致命的 PMCT，1、3、5 年生存率分别为 47%、16%和 11%和 51%、26%和 23%，而相应的淋巴瘤分别为 59%、41%和 34%（对数秩检验 P<0.001）。与具有相似组织病理学的心脏外疾病患者相比，心脏淋巴瘤和肉瘤患者更年轻，生存情况更差（P<0.001）。
PMCT 极为罕见，且预后仍较差。在过去的 5 十年中，诊断为 PMCT 的患者的发病率和生存率似乎有所提高。与具有相似组织病理学的心脏外癌症患者相比，PMCT 患者通常更年轻，生存情况更差。
