Cardiac angiosarcoma: two cases and a review of the literature

Abstract

Background: Primary cardiac tumours are rare, and primary malignant cardiac tumours even rarer. Of these, cardiac angiosarcomas are uncommon and, until recently, almost invariably diagnosed at the time of autopsy, primarily because the symptoms are initially nonspecific and do not become manifest until the tumour is advanced.

Methods: Two patients, who presented in quite different manners and were diagnosed at autopsy and at open surgical biopsy, are presented. The literature on cardiac angiosarcomas is reviewed critically, with emphasis on presentation and morphology.

Results: This review of the literature shows that, with increasing availability of newer diagnostic tools, especially noninvasive ones, diagnosis of this rare lesion can be made at an early stage and confirmed at cardiac biopsy or cardiac surgery. Unfortunately, so far, the results remain virtually uniformly poor, though occasionally survival at up to 53 months has been reported.