Characteristics and Survival of Malignant Cardiac Tumors: A 40-Year Analysis of Over 500 Patients
Background—To investigate the incidence, histopathology, demographics and survival associated with primary malignant cardiac tumors (PMCTs).
Methods and Results—We queried the Surveillance, Epidemiology and End-Results (SEER) 18-registry from National Cancer Institute for all PMCTs diagnosed from 1973 to 2011. We describe PMCT histopathology and incidence, comparing characteristics and survival of these patients, to those with extra-cardiac malignancies of similar histopathology. From a total of 7,384,580 cases of cancer registered in SEER, we identified 551 (0.008%) PMCTs. The incidence of PMCT diagnosis is 34 cases per 100 million persons and has increased over time: 25.1 (1973-1989), 30.2 (1990-1999) and 46.6 (2000-2011). Most patients are female (54.1%), 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 was 46%, 22%, and 17% and has improved over the eras with (1-, 3-, 5-year) of 32%, 17%, 14% (1973-1989) to 50%, 24%, 19% (2000-2011) (p=0.009). Cardiac sarcomas and mesotheliomas are the most lethal PMCTs, with 1-, 3-, 5- year survival of 47%, 16%, and 11%, and 51%, 26%, and 23%, respectively, compared with lymphoma 59%, 41%, and 34%, respectively (log rank test p<0.001). Patients with cardiac lymphomas and sarcomas are younger and have worse survival than patients with extra-cardiac disease of similar histopathology (p<0.001).
Conclusions—PMCTs are extremely rare and continue to be associated with poor prognosis. Over the past five decades, the incidence and survival of patients diagnosed with PMCT appears to have increased. Compared to those with extra-cardiac cancers of similar histopathology, patients with PMCTs are often younger and have worse survival.
- Received March 10, 2015.
- Revision received September 18, 2015.
- Accepted October 2, 2015.