Korean Circ J.  2010 Feb;40(2):86-89. 10.4070/kcj.2010.40.2.86.

Primary Cardiac Angiosarcoma Presenting With Cardiac Tamponade

• Kim DM
• Hong JH
• Kim SY
• Yoo KD
• Seo JY
• Rhee KJ
• Kim BO
• Goh CW
• Park KM
• Kim JH
• Huh JH
• Kim HJ
• Byun YS

• KMID: 2297944
• DOI: http://doi.org/10.4070/kcj.2010.40.2.86

Abstract

Primary cardiac angiosarcoma is a very rare disease with a poor prognosis. We report a case of a patient with a primary cardiac angiosarcoma who presented with cardiac tamponade; the angiosarcoma was successfully resected surgically.

Keyword

Cardiac tumor; Angiosarcoma; Hemopericardium

MeSH Terms

Cardiac Tamponade
Heart Neoplasms
Hemangiosarcoma
Humans
Pericardial Effusion
Prognosis
Rare Diseases

Figure

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  Fig. 1 Round, increased cardiac silhouette and right costophrenic angle blunting on chest radiography.

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  Fig. 2 Chest CT (A) and transthoracic echocardiography (B) shows a large and inhomogeneous, enhancing mass in the right atrium (arrow) and a massive amount of pericardial and right pleural effusion.

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  Fig. 3 Transesophageal echocardiography shows a primary cardiac mass without involvement of the inferior and superior vena cava. IVC: inferior vena cava, SVC: superior vena cava, RA: right atrium, LA: left atrium.

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  Fig. 4 A: a resected protruding mass (6.8×6.5×2.5 cm) into the right atrial chamber is present at the atrial wall. B: the cut surface is diffusely hemorrhagic with a gray-white fleshy solid portion. C: the tumor is composed of oval-to-spindle cells with intracytoplasmic red blood cells. There are frequent mitoses (> 10/10 HPF). D: the tumor cells are positive for CD34 immunohistochemical staining.

Reference

1. Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. 1996. 77:107.

2. Hanfling SM. Metastatic cancer to the heart: review of the literature and report of 127 cases. Circulation. 1960. 22:474–483.

3. Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer. 1992. 69:387–395.

4. Simpson L, Kumar SK, Okuno SH, et al. Malignant primary cardiac tumor. Cancer. 2008. 112:2440–2446.

5. Meng Q, Lai H, Lima J, Tong W, Qian Y, Lai S. Echocardiographic and pathologic characteristics of primary cardiac tumors: a study of 149 cases. Int J Cardiol. 2002. 84:69–75.

6. Dawson WB, Mayo JR, Muller NL. Computed tomography of cardiac and pericardial tumors. Can Assoc Radiol J. 1990. 41:270–275.

7. Shin MS, Kirklin JK, Cain JB, Ho KJ. Primary angiosarcoma of the heart: CT characteristics. AJR Am J Roentgenol. 1987. 148:267–268.

8. Smith DN, Shaffer K, Patz EF. Imaging features of nonmyxomatous primary neoplasms of the heart and pericardium. Clin Imaging. 1998. 22:15–22.

9. Putnam JB Jr, Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA. Primary cardiac sarcomas. Ann Thorac Surg. 1991. 51:906–910.

10. Min DJ, Kang DH, Seung KB, et al. A primary cardiac angiosarcoma. Korean Circ J. 1995. 25:704–709.

11. Kim JS, Song SG, Ko WS, et al. A case of primary right atrial angiosarcoma manifested with cardiac tamponade. J Korean Soc Echocardiogr. 2004. 12:36–38.

12. Llombart-Cussac A, Pivot X, Contesso G, et al. Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience. Br J Cancer. 1998. 78:1624–1628.

13. Alvarez JM, Yew MK, Rajesh B, Ireland M. Cardiac angiosarcoma: too little known, too late treatment or just too bad a tumour? Heart Lung Circ. 2001. 10:30–34.

14. Donsbeck AV, Ranchere D, Coindre JM, Le Gall F, Cordier JF, Loire R. Primary cardiac sarcomas: an immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology. 1999. 34:295–304.

15. Talbot SM, Taub RN, Keohan ML, Edwards N, Galantowicz ME, Schulman LL. Combined heart and lung transplantation for unresectable primary cardiac sarcoma. J Thorac Cardiovasc Surg. 2002. 124:1145–1148.