Right atrium angiosarcoma with feeding vessels from right coronary artery: a case report

BACKGROUND: Primary cardiac angiosarcoma is a rare primary cardiac malignancy. Biopsy of such vascular-rich tumours may result in serious complications. CASE SUMMARY: This is a case of a 43-year-old woman who presented with syncope. According to clinical history, she initially had massive pericardial effusion, with an uncertain aetiology. Multimodality imaging at our hospital revealed a cauliflower-like tumour in the right atrium. Coronary angiography results confirmed multiple feeding vessels from the right coronary artery to the tumour. Thoracoscopic biopsy resulted in a massive bleeding requiring haemostasis via thoracotomy. Histopathological examination of the specimen showed an angiosarcoma with atypical cells and spindle cells in a myxomatous background. Treatment with systemic targeted therapy and chemotherapy was initiated, and the patient is still under active treatment. DISCUSSION: Cardiac angiosarcomas most commonly arise from the right atrium and may be hard to detect with transthoracic echocardiography. Biopsy of primary cardiac angiosarcomas requires careful planning because they are highly vascularized. Currently, no guidelines regarding the treatment of such tumours exist, and a multidisciplinary treatment is needed.