Right Atrial Angiosarcoma with Severe Biventricular Dysfunction and Massive Pericardial Effusion
This paper presents the case of a 35 year-old woman with symptoms of heart failure from the last month. A physical examination at admission showed paleness, dyspnea, peripheral edema and fatigue. In a two-dimensional echocardiography and transesophageal echocardiography, normal thickness but severe left and right ventricular dysfunction with severe pericardial effusion and thickened pericardium were found. In the enlarged right atrium, an oval-shaped structure was found with features of continuity with lateral right atrial wall and also a bulging of the structure through the orifice of the tricuspid valve to the right ventricle. In the echocardiography, we did not saw any blocking of the tricuspid valve or the inflow from inferior vena cava (IVC) or superior vena cava (SVC) or coronary sinus. On the basis of the echocardiography examination and clinical presentation, tentative diagnosis of the right atrium myxoma was made. A coronary angiography revealed normal coronary arteries and no feeding of tumor by branch of right coronary artery (RCA). Surgical removal of the tumor was performed without complication. The histopathological examination confirmed the diagnosis of angiosarcoma. In the follow-up echocardiography carried out after three months, severe left ventricular (LV) and right ventricular (RV) dysfunction continued and was demonstrated. Magnetic resonance imaging revealed no lymphadenopathy or re-growth of the tumor in the mediastinum or pericardium.
Janigan DT, Husain A, Robinson Na. Cardiac angiosarcomas. A review and a case report. Cancer 1986; 57(4):852-9.
Silverman NA. Primary cardiac tumor. Ann Surg 1980; 191(2):127-38.
Herrmann MA, Shankerman RA, Edwards WD, Shub C, Schaff HV. Primary cardiac angiosarcoma: a clinicopathologic study of six cases. J Thorac Cardiovasc Surg 1992; 103(4):655-64.
McFadden PM, Ochsner JL. Atrial replacement and tricuspid valve reconstruction valve reconstruction after angiosarcoma resection. Ann Thorac Surg 1997;64(4):1164-6.
Kaminaga T, Takeshita T, Kimural. Role of magnetic resonance imaging for evaluation of tumors in the cardiac region. Eur Radiol 2003; 13(Suppl 4):L1-10.
Randall MB, Geisinger KR. Angiosarcoma of the heart:pericardial fluid cytology. Diagn Cytopathol 1990; 6(1):58-62.
Nayar S, Nayar PG, Cherian K. Angiosarcoma presenting as syncope. Asian Cardiovasc Thorac Ann 2008; 16(2):154-6.
Glancy DL, Morales JB Jr, Roberts WC. Angoisarcoma of the heart. Am J Cardio 1968;21(3):413-9.
Amonkar GP, Deshpande JR. Cardiac angiosarcoma. Cardiovasc Pathol 2006; 15(1):57-8.
Shapiro LM. Cardiac tumours:diagnosis and management..Heart 2001; 85(2):218-22.
Adem C, Aubry MC, Tazelaar HD, Myers JL. Metastatic angiosarcoma masquerading as diffuse pulmonary hemorrhage: clinicopathologic analysis of 7 new patients. Arch Pathol Lab Med 2001; 125(12):1562-5.
Kontogiorgi M, Exarchos D, Charitos C, Floros I, Rontogianni D, Roussos C, Routsi C. Primary right atrium angiosarcoma mimicking pericarditis. World J Surg Oncol 2007; 5:120.
Herrmann MA, Shankerman RA, Edwards WD, Shub C, Schaff HV. Primary cardiac angiosarcoma: a clinicopathologic study of six cases. J. Thorac Cardiovasc Surg 1992; 103(4):655-64.
Kakizaki S, Takagi H, Hosaka Y. Cardiac angiosarcoma responding to multidisciplinary treatment. Int J Cardiol 1997; 62(3):273-5.
Freudenberg LS, Rosenbaum SJ, Schulte-Herbrüggen J, Eising EG, Lauenstein T, Wolff A, Bockisch A. Diagnosis of a cardiac angiosarcoma by fluorine-18 fluordeoxyglucose positron emission tomography. Eur Radiol 2002; 12 Suppl 3:S158-61.
Huang J, Bouvette MJ, Zhou J, Dwyer GJ 3rd, Bhopatkar S, Bhatia A. A large angiosarcoma of the right atrium. Anesth Analg 2009; 108(6):1755-7.
Vaitkus PT, Hermann HC, LeWinter MM. Treatment of malignant pericardial effusion.JAMA 1994; 272(1):59-64.
Ayyaz Sultan, Ahmed Amour, Sarfraz Khan. Cardiac angiosarcoma presenting with death due to cardiac perforation. Br J Cardiol 2006; 13:213-5.
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