Radical multidisciplinary approach to primary cardiac sarcomas

Objectives: Primary cardiac sarcomas are extremely rare, but aggressive, tumours. The median survival with conventional treatment is 6-12 months. Recent data suggest that a radical multidisciplinary approach may improve patient outcome. We sought to evaluate our institutional experience with these tumours. Methods: A multidisciplinary cardiac tumour programme was established 3 years ago based on the experience and support of our collaborating institution. Treatment consisted of pre- and postoperative chemotherapy, complete (R0) resection of the tumour with structural reconstruction and radiation therapy in selected cases. Left atrial tumours were resected using the cardiac autotransplantation technique. Bovine pericardium was used to reconstruct free-chamber walls or the septum. Valves were replaced by bioprostheses. A variety of autologous, allogeneic and synthetic vascular grafts were used to reconstruct the aorta, pulmonary arteries (PAs) and coronary arteries. Results: Seven patients (3 males), age 51 ± 11 years (35-63), underwent eight operations. Tumour sites were PAs in 2 patients, left atrium in 3, right atrium in 2 and both great vessels in 1. Complete resection was achieved in all cases. There was no operative mortality. Two patients required implantation of a permanent pacemaker. Median survival was 24 months. Three patients died of metastatic disease and 1 sudden death 7, 23, 31 and 33 months after diagnosis. Three patients are alive at 2, 8 and 33 months, in functional Class I or II. One patient developed tumour recurrence and 2 have no evidence of disease. Conclusions: A radical multidisciplinary approach to cardiac sarcomas consisting of multimodality treatment and complex, technically demanding surgery, is safe and markedly improves (doubling) patient survival.