Brain metastases are common in patients with primary cardiac sarcoma, and location of the sarcoma on the heart is associated with disease histology, as well as the likelihood of developing brain metastases. The results of this study were published in Sarcoma.

Primary cardiac sarcoma is the most common type of primary cardiac tumor, although these tumors are very rare. Hence, information regarding the clinicopathologic characteristics and clinical outcomes of patients with primary cardiac sarcomas is limited.

In this retrospective, single-institution study, 39 patients with primary cardiac sarcoma, excluding those with sarcoma of the pulmonary vasculature or great vessels, treated at the University of Michigan between 1992 and 2017 were identified using the University of Michigan Electronic Medical Record Search Engine.

Median age at diagnosis was 41 years, and median tumor size was 6.5 cm. The most frequent symptom at diagnosis was dyspnea, observed in 74% of patients. Thirty-one percent of patients had cardiac tamponade at diagnosis, the majority of whom had right atrial tumors. In addition, pericardial effusion and pulmonary hypertension was observed in 51% and 21% of patients at diagnosis, respectively.

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In the overall group of patients, tumor location was fairly equally divided between the right (41%) and left sides of the heart (46%), with most of the remaining tumors occurring in the pericardium (13%). The most common disease histologies were angiosarcoma (36%), high-grade undifferentiated pleomorphic sarcoma (UPS; 26%), and leiomyosarcoma (13%). Interestingly, angiosarcomas were more likely to be found on the right side of the heart, while UPS and leiomyosarcomas were more commonly located on the left side of the heart.  

Almost half of patients (46%) had metastatic disease at the time of diagnosis. Although 10.6% of patients had brain metastases at diagnosis, nearly one-third of patients developed metastases to the brain at some point in their clinical course. Notably, the majority of brain metastases occurred in patients with left-sided tumors and were most commonly of UPS histology.

Nearly two-thirds of patients underwent surgical resection although an R0 resection was achieved in only 12%, all of whom had left-sided tumors. Radiation therapy to the primary tumor and the brain was administered to 7.7% and 20.5% of patients, respectively. Approximately 72% of patients received chemotherapy (eg, first-line doxorubicin plus ifosfamide or first- or second-line gemcitabine plus docetaxel), mostly in the postsurgical setting.  

Median overall survival (OS) for those treated with and without surgery was 14.0 months and 8.2 months, respectively. Furthermore, median OS was 14.0 months and 2.4 months in patients who did or did not receive chemotherapy, respectively (P =.0001).

Limitations of this study include its retrospective design, as well as the possibility that patients receiving chemotherapy had better performance status and organ function.

“Based on our findings, we recommend brain imaging at the time of primary cardiac sarcoma diagnosis given the incidence of brain metastases. Furthermore, our results suggest a role for aggressive multimodal therapy including resection and systemic therapy, even in the metastatic setting, to improve outcomes,” the researchers concluded.

Reference

Siontis BL, Zhao L, Leja M, et al. Primary cardiac sarcoma: A rare, aggressive malignancy with a high propensity for brain metastases [published online March 10, 2019]. Sarcoma. doi: 10.1155/2019/1960593