Primary Cardiac Sarcoma

doi:10.1016/j.athoracsur.2010.03.065

The Annals of Thoracic Surgery

Volume 90, Issue 1, July 2010, Pages 176-181

https://doi.org/10.1016/j.athoracsur.2010.03.065 Get rights and content

Background

The presentation, management, and outcome of patients with primary cardiac sarcomas are not well defined. Furthermore, the role of adjuvant therapy has not been delineated in the management of primary cardiac sarcomas.

Methods

Patients with primary cardiac sarcoma and noncardiac sarcoma, diagnosed between 1988 and 2005, were identified in the Surveillance, Epidemiology, and End Results (SEER) database. Clinical characteristics and outcomes of primary cardiac sarcoma were defined and compared with the characteristics of noncardiac sarcomas. Univariate and multivariate methods were used to identify factors associated with primary cardiac sarcoma survival.

Results

Compared with noncardiac sarcomas, primary cardiac sarcomas were found to occur in a younger age group and were more likely to present with advanced disease. Primary cardiac sarcomas were ten times more likely to be vessel-derived (eg, angiosarcoma), comprising almost half of all cases. Median overall survival for cardiac sarcoma patients was 6 months whereas that for noncardiac sarcoma patients was significantly longer at 93 months (p < 0.001). Furthermore, cardiac sarcoma patients who underwent surgery had a median survival of 12 months whereas those who did not undergo surgery had a median survival of 1 month (p < 0.001).

Conclusions

Cardiac sarcomas are a distinct, rare subset of soft tissue sarcomas with a poor prognosis. Surgery continues to be the central component of successful management. Future clinical efforts should be directed at developing approaches to permit safe radical excision and, potentially, developing effective adjuvant therapy.

Section snippets

Study Population

The SEER-17 registries data set of the US National Cancer Institute (April 2008 release) was used to identify patients diagnosed with primary cardiac sarcomas and noncardiac sarcomas between January 1, 1988, and December 31, 2005. Unspecified neoplasms, epithelial neoplasms, squamous cell carcinomas, adenomas, adenocarcinomas, paragangliomas, glomus tumors, and gliomas were excluded. Additional inclusion criteria consisted of known age, sex, race, and tumor stage. A total of 210 patients met

Results

The study population consisted of 210 patients diagnosed with primary cardiac sarcoma and 24,404 patients with noncardiac sarcoma. Patient characteristics, tumor grade, SEER stage, and histologic type are shown for both cardiac sarcoma and noncardiac sarcoma patients in Table 1. Although both cancers predominated in patients younger than 65, primary cardiac sarcoma patients were far more likely to present at a younger age. At the time of diagnosis, an equal number of primary cardiac sarcoma

Comment

Although primary cardiac sarcomas occur over a wide age range, they are more likely to present in patients younger than 65 years old. Previous retrospective series have also found that the median age of cardiac sarcoma patients was in the 40s with the majority of cases diagnosed in the fourth and fifth decades of life [6, 7, 8, 9, 10, 11, 12]. In terms of sex, we found a slight male predominance. Most previous studies have found that malignant cardiac tumors are equally prevalent in both sexes [

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Original articleAdult cardiacPrimary Cardiac Sarcoma

Background

Methods

Results

Conclusions

Section snippets

Study Population

Results

Comment

Am J Cardiol

Ann Thorac Surg

Hum Pathol

J Thorac Cardiovasc Surg

J Heart Lung Transplant

Chest

J Thorac Cardiovaxc Surg

Neoplasms metastatic to the heart: review of 3314 consecutive autopsies

Am J Cardiovasc Pathol

Original article
Adult cardiac
Primary Cardiac Sarcoma