Purpose: To identify the clinicopathologic characteristics, treatments, and outcomes of a series of patients with primary cardiac angiosarcoma (AS).
Methods: This retrospective case series was set in a tertiary referral center with a multidisciplinary clinic. Consecutive patients with institutionally confirmed pathologic diagnosis of cardiac AS from January 1990 to May 2011 were reviewed. Main outcome measures included patient demographics, tumor characteristics, management strategies, disease response, and survival.
Results: Data from 18 patients (78 % male) were reviewed. Sixteen patients (89 %) had AS originating in the right atrium. At diagnosis, eight patients (44 %) had localized/locally advanced disease and ten patients (56 %) had metastatic disease. Initial treatment strategies included resection (44 %), chemotherapy (39 %), and radiotherapy (11 %). Of the eight patients with localized/locally advanced AS, two underwent macroscopically complete resection with negative microscopic margins, one underwent macroscopically complete resection with positive microscopic margins, one underwent macroscopically incomplete resection, two received chemotherapy followed by surgery and intraoperative radiotherapy, one received chemotherapy alone, and one died before planned radiotherapy. Median follow-up was 12 months. Median overall survival (OS) was 13 months for the entire cohort; median OS was 19.5 months for those presenting with localized/locally advanced AS and 6 months for those with metastatic disease at presentation (p = 0.08). Patients who underwent primary tumor resection had improved median OS compared with patients whose tumors remained in situ (17 vs. 5 months, p = 0.01).
Conclusions: Cardiac AS is associated with poor prognosis. Resection of primary tumor should be attempted when feasible, as OS may be improved. Nevertheless, most patients die of disease progression.