Ann Thorac Surg 2006;82:1576
© 2006 The Society of Thoracic Surgeons
Correspondence
Familial Cardiac Angiosarcoma
Ingeborg M. Keeling, MD,
Ferdinand Ploner, MD,
Bruno Rigler, MD
Divisions of Cardiac Surgery and Oncology, Medical University of Graz, Auenbruggerplatz 29, Graz, 8036 Austria
(Email: ingeborg.keeling{at}klinikum-graz.at).
To the Editor:
We read with interest Iskander and colleagues' article [1] on left atrial sarcoma resection and autotransplantation, as we encountered 2 brothers with cardiac angiosarcoma within a 13-year interval. Case 1 was only diagnosed on autopsy after cardiac resuscitation in a referred hospital. Case 2, a 39-year-old man, was admitted to the hospital with incipient pericardial tamponade seen on transthoracic echocardiography (TTE). Multi-slice computed tomography and transesophageal echocardiography suggested the presence of a tumor mass (4 x 5 cm) originating from the right atrium (RA) near the tricuspid valve, which also infiltrated the pericardium, left atrium, and the angle between the basis of the mitral valve (MV) and the aortic root. On normothermic bypass, a wall-perforating RA tumor was immediately resected. Tumor staging confirmed absence of systemic metastases. However, 1 month postoperatively TTE showed a recurrent lesion on the level of the atrioventricular valves extending to the MV. Lacking sufficient data regarding systemic treatment of angiosarcoma, palliative mono-chemotherapy with paclitaxel at a dosage of 175 mg/m2 (given every 3 weeks) was started. The maximum response after four cycles of therapy was a short-lasting stabilization; the patient died 5 months postoperatively from tumor progression.
This is the second report on familial cardiac angiosarcoma [2]. All four known familial angiosarcomas originated from the right atrium and showed excessively rapid progression. Familial angiosarcoma patients died within 9 months (mean survival time, 4 months) after surgery compared with a mean survival of 11 months and a 5-year survival rate of 14% in sporadic angiosarcoma [3].
We agree that complete resection remains a cornerstone and important determinant of local recurrence and duration of disease-free survival. The only chance to achieve an acceptable long-term survival may be a more radical approach, even radical resection crossing anatomical boundaries of valves, coronary arteries, and conduction system, sometimes facilitated by an ex situ procedure or autotransplantation [1]. Consecutive reconstruction may be performed using autologous or homologous material, additional valve replacement, coronary artery bypass grafting, and cardiomyoplasty. Further options may be the temporary implantation of a ventricular assist device or a total artificial heart for support of a failing heart after radical tumor surgery. Orthotopic heart transplantation or heart-lung transplantation, using a modified immunosuppressive regime, may be an option in selected cases. However, multicentric, excessively infiltrative angiosarcomas may not be operable. Furthermore, there is no evidence available concerning additional benefit from adjuvant systemic treatment after curative intended resection of the primary tumor.
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References
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- Iskander SS, Nagueh SF, Ostrowski ML, Reardon MJ. Growth of a left atrial sarcoma followed by resection and autotransplantation Ann Thorac Surg 2005;79:1771-1774.[Abstract/Free Full Text]
- Casha AR, Davidson LA, Roberts P, Unnikrishnan N. Familial angiosarcoma of the heart J Thorac Cardiovasc Surg 2002;124:392-394.[Free Full Text]
- Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart Cancer 1992;69(2):387-395.[Medline]
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