Prognosis poor for both men and women, although survival odds favored patients with lymphoma

Survival rates in patients with primary malignant cardiac tumor (PMCT) were not different between men and women, a longitudinal analysis of a large database found; however, men tended to be diagnosed with the malignancy at an earlier age than women, the same analysis showed.

Having explored the Surveillance, Epidemiology and End Results (SEER) 18 database between 1973 and 2015 for patients diagnosed with PMCT, researchers identified 327 men and 367 women with PMCTs out of a total of 7,384,580 cases of cancer registered in SEER.

Median overall survival (OS) for patients with PMCT was 16 months for men and 14 months for women, Daniel Antwi-Amoaberg, MD, University of Nevada Reno School of Medicine, Reno, Nevada, and colleagues reported in the Journal of the American Heart Association.

Overall survival was longest among patients diagnosed between 18 and 50 years of age, regardless of gender, but men between 51 to 65 years of age at the time of diagnosis had better survival odds than those in other age groups (P=0.0103), investigators added.

Nevertheless, only half of the patients identified in the current analysis were alive at the end of 25 months, a sign that PMCT was still associated with a poor prognosis across a span of more than four decades.

“PMCTs are exceedingly rare, accounting for only 0.008% of all malignancies reported in the SEER database between 1973 and 2011,” Antwi-Amoaberg and colleagues observed.

“We did not observe any significant sex disparity in long-term survival [and] compared with extracardiac cancers, patients with PMTCs have worse outcomes [with a] 1-year survival rate [of] approximately 10% without surgical resection,” they noted.

The greatest proportion of patients diagnosed with PMCT was between 18 and 50 years of age. “The incidence of PMCT decreased after age 50 years,” investigators noted, “[while a]pproximately 78% of all patients diagnosed with PMCT were white,” they added.

Data were available for 3 histologic subtypes of PMCT — lymphoma, sarcoma, and mesothelioma. “Sarcoma was the most common type of PMCT (~60%) followed by lymphoma, mesothelioma, and others,” researchers reported.

There were no observable differences in the distribution of tumor types between men and women, although survival was highest for patients with lymphoma and lowest for patients diagnosed with mesothelioma, they added. However, patients with lymphoma-type PMCT had the best overall survival rates, at a mean of roughly 40% at 5 years.

Approximately half of women diagnosed with PMCT underwent surgical resection, as did 43% of men, but only a small proportion (11%) of all patients with PMCT were treated with both surgical resection and radiation.

The low use of radiation therapy might reflect physicians’ reluctance to use radiation due to radiation-induced cardiac toxicity, the authors speculated. Moreover, sarcoma is not highly sensitive to radiation, so moderate levels of radiotherapy would likely be minimally effective.

“[Since m]ore than a quarter of men with primary malignant cardiac tumors are diagnosed before the age of 50 years…a prompt and thorough evaluation should be performed in young men presenting with suspicious symptoms,” the authors suggested.

“[And] improved outcomes can be achieved through early detection and surgical intervention,” they concluded.

Commenting on the study, editorialist Rajeev Gupta, MD, Mediclinic Al-Jowhara/Al Ain Hospitals, United Arab Emirates, and colleagues pointed out that it “would have been interesting to note response to therapy among those with sarcomas (how many could be excised and cured or recurred?) and lymphomas (how many responded to the treatment?).” However, despite these limitations, “this study provides important data on primary malignant cardiac tumors,” they wrote.

As with findings from an earlier registry-based study, this study confirms that PMCT is a rare disease and that it still has a poor prognosis, the editorialists emphasized.

However rare, “[its] existence reminds us to be aware and to explore/investigate a cardiac mass/growth or rapidly developing pericardial effusion with/without cardiac tamponade or treatment-resistant rapidly progressing heart failure,” Gupta and colleagues advised.

“A remote possibility of subtle mass/growth while investigating a patient with unexplained arrhythmia or conduction disturbances should also be kept in mind at the time of echocardiographic study/CMR/MDCT study,” they suggested.

  1. There has been no difference in survival odds between men and women diagnosed with primary malignant cardiac tumor (PMCT) over the past 4 decades, with a median overall survival of approximately 15 months for both genders.

  2. The most common type of PMCT diagnosed in both men and women was sarcoma but those diagnosed with lymphoma had the best overall survival.

Pam Harrison, Contributing Writer, BreakingMED™

Antwi-Amoabeng and Gupta disclosed no relevant relationships.

Cat ID: 120

Topic ID: 78,120,730,914,120,935,192