Successful surgical treatment for primary cardiac angiosarcoma: a case report.

BACKGROUND

Primary cardiac angiosarcomas are extremely rare and their prognosis is poor. Surgical resection is the first-line treatment; however, no clear standard of care has been clearly established because of the rarity of these tumors.

CASE PRESENTATION: A 61-year-old man who had presented with dyspnea on exertion was referred to our hospital. Contrast-enhanced computed tomography revealed massive pericardial effusion and a 40-mm enhanced mass adherent to the anterior wall of the right atrium and involving the right coronary artery. Having diagnosed the mass as a cardiac tumor, we resected the mass under the guidance of epi-cardiac echocardiography guidance, which showed continuity between the tumor and the right atrium, reconstructed the right atrial free wall with a bovine pericardial patch, and performed coronary artery bypass grafting to the right coronary artery using the great saphenous vein. The right atrial wall was resected with adequate tumor-free margin. On the right ventricular side, we resected the right atrial wall 1 cm from the tumor, 2 cm from the atrioventricular groove. Because hemodynamic deterioration occurred after aortic declamping, intra-aortic balloon pumping and veno-arterial extracorporeal membrane oxygenation were instituted. Postoperatively, circulatory support devices were removed safely, and the patient was discharged on the 25th postoperative day. Histopathological examination of the surgical specimens resulted in a diagnosis of angiosarcoma, with positive surgical margins. Chemotherapy and radiotherapy (69 Gy in 30 fractions) were therefore initiated after discharge. To date, the patient has been alive and well with no recurrence of tumor for 4 years and 10 months since surgery.

DISCUSSION: This case study suggests the usefulness of multimodality treatment comprising surgical resection and adjuvant therapy, for cardiac angiosarcoma.