Characteristics and Survival of Malignant Cardiac Tumors
What is the incidence, histopathology, demographics, and survival associated with primary malignant cardiac tumors (PMCTs)?
The study authors queried the Surveillance, Epidemiology and End-Results (SEER) 18-registry from the National Cancer Institute for all PMCTs diagnosed from 1973 to 2011. They described PMCT histopathology and incidence, and compared characteristics and survival of these patients to those with extracardiac malignancies of similar histopathology.
The study authors found that from a total of 7,384,580 cases of cancer registered in SEER, 0.008% (n = 551) were 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 white (78.6%) and female (54.1%), with median age at diagnosis of 50 years. Most patients are diagnosed with tissue sample (96.8%) and the most common PMCTs are sarcomas (n = 357, 64.8%), followed by lymphomas (n = 150, 27%) and mesotheliomas (n = 44, 8%). Patients with cardiac lymphomas and sarcomas are younger and have worse survival than patients with extracardiac disease of similar histopathology (p < 0.001). Although use of chemotherapy is not documented in this registry, 44% had surgery, 19% of patients received radiation, and 10.2% received both surgery and radiation.
Survival data were available for 516 (93.6%) patients. After a median follow-up of 80 months, 413 patients had died. The 1-, 3-, and 5-year survival was 46%, 22%, and 17%, respectively, and has improved over the eras (1-, 3-, 5-year survival) 32%, 17%, and 14% (1973-1989) to 50%, 24%, and 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% sarcomas, respectively, compared with lymphoma 59%, 41%, and 34%, respectively (log rank test p < 0.001).
The authors concluded that PMCTs are extremely rare and continue to be associated with poor prognosis. However, the incidence and survival of patients diagnosed with PMCT over the past five decades appears to have increased. Compared to those with extracardiac cancers of similar histopathology, patients with PMCTs are often younger and have worse survival.
This is an important study because it gives an overview of these rare tumors. The findings of this study that the incidence of PMCTs is increasing, suggest that the quest for therapy, particularly for sarcomas and mesotheliomas, is urgent.
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