Primary cardiac angiosarcoma: a fatal disease

Abstract

A 42-year-old man with a cardiac tamponade underwent an urgent pericardiotomy that showed tumoral tissue, covering the surface of the right atrium. The tumor was then partially excised, and the histological examination revealed the presence of a moderately-differentiated angiosarcoma. The patient was then referred to the oncology unit and scheduled for a chemotherapy schedule including Epirubicin (60 mg/m(2), on days 1 and 2) plus Ifosfamide (2000 mg/m(2), on days 1 to 3) and Uromitexan (2000 mg/m(2) at hours 0, 4, 8 after IFO). All drugs were administered every three weeks. After two cycles, a restaging work-up revealed a partial remission. The treatment was continued for another two cycles. A new evaluation by cardiac MRI evidenced a local and distant (lung) progression of disease. The patient died after three months. This paper confirms that cardiac angiosarcoma is a fatal disease, and the prognosis is usually 6-11 months from time of diagnosis.

Figure 1

(a) (colouring H&E 10×) Angiosarcoma moderately differentiated. (b) (colouring H&E 40×) Greater enlargement angiosarcoma moderately differentiated. (c) (20×) Immunohistochemical investigation tumor cells were positive for CD 31. (d) (20×) Immunohistochemical investigation tumor cells were positive for CD34. (e) (20×) Immunohistochemical investigation tumor cells were positive for FVIII rAg. (f) (40×) Positive for Mib-1 60%.

Figure 2

Voluminous left pleural effusion. Large mass extending from the free wall of the right atrium to the anterior mediastinum beside the superior vena cava and ascending aorta and above until the right pulmonary artery.