Primary angiosarcoma of the heart mimicking a left atrial myxoma.

Abstract

Sarcomas as a group are the second most common type of primary tumour of the heart.' Angiosarcoma is, however, an unusual form of tumour within this group and is rarely diagnosed during life.2 It is almost exclusively a tumour of the right atrium and pericardium, patients presenting with symptoms of right heart inflow obstruction and pericardial disease.3 We present here a case of primary angiosarcoma of the heart causing obstruction of the mitral valve. Two dimensional echocardiography suggested the presence of a left atrial myxoma. Resection was attempted. The histological diagnosis was made from tissue obtained at surgery. Case reportA 48 year old unemployed farm worker presented to his local hospital with ill health of several weeks' duration, weight loss, anorexia, and vomiting. Initial examination showed wasting and hepatomegaly and investigations showed anaemia (haemoglobin 11 g/dl) and an erythrocyte sedimentation rate of 120 mm in one hour. Further investigation was advised but the patient declined it. One month later he returned after an episode of central chest pain and severe dyspnoea. In the intervening month he had noticed increasing breathlessness. On examination there were signs of biventricular failure, a pericardial rub, and an apical diastolic murmur. Ultrasound examination showed a mass in the left atrium.The patient was transferred to the regional cardiac unit, where examination showed an ill, thin man with peripheral oedema. His pulse was 110 beats/min and regular, his blood pressure 110/90 mm Hg. The central venous pressure was raised and the left ventricular impulse tapping. There was a loud pericardial rub and a loud mitral diastolic murmur. The liver was enlarged 6 cm below the costal margin. The electrocardiogram showed sinus rhythm with evidence of left atrial hypertrophy. The QRS complexes were of small amplitude and there were widespread nonspecific ST segment and T wave changes. There were bilateral pleural effusions on the chest radiograph. The ascending aorta appeared prominent. appeared to be indented by the "effusion" in ventricular systole. The echo free space extended anterior to the right ventricular outflow tract but no other abnormality of the great vessels was seen. A diagnosis of left atrial myxoma and pericardial effusion was made. The latter seemed remarkable, but in the presence of severe biventricular cardiac failure, pleural effusions, and hypoalbuminaemia this was attributed to his general condition. The patient was referred for resection of the tumour.At operation, no pericardial effusion was found but the pericardial cavity was full of blood clot. There were extensive pericardial adhesions. There was a large extracardiac tumour occupying the space between the superior vena cava and the aorta. The tumour was distorting and eroding the right atrium but there was no tumour in the right heart cavities. The right atrium and pulmonary artery were enlarged. A large endothelialised tumour was found occupying the entire left atrial cavity, almost totally obstructing the ...

Pedunculated Left Atrial Rhabdomyosarcoma Simulating a Prolapsing Myxoma: Limitations of Echocardiography

“…A similar experi ence was reported by Ormerod et al [11], Sorbe et al [13], Hammond et al [14], Winer et al [15], Shah et al [16], Olds et al [20], and Nzayinambaho et al [22] who all observed predunculated primary cardiac sarcomas (angiosarcoma, chondrosarcoma, osteosarcoma, fibrous histiocytoma, mesothelioma, liposarcoma) initially thought to be atrial myxomas (left or right) by clinicoechocardiographic examination. Rosman et al [23] have reported right and left atrial nonprolapsing rhabdomyosarcomas, and Schmaltz and Apitz [21] described a primary rhabdomyosarcoma in the pediatric age group.…”

“…The presence of pericar dial effusion may be a clue to the diagnosis of malignan cy. Metastatic lesions on the surface of the heart may be observed and tumor infiltration of the heart walls visual ized; however, the extent of intramural growth may not be recognized or heavily underestimated by 2D, particu larly when the images are less than optimal [8,11]. Mich et al [19] stressed the significance of tumor extension into pulmonary veins and an unusual site of tumor ori gin as significant clues to the diagnosis of malignancy.…”

“…Third, the specificity of echocardiography is less than 100%, since the échocardiographie pictures of LAM can be simulated not only by vegetations and thrombotic masses, but also by primary nonmyxomatous neoplasms with pedunculated or sessile attachment or by secondary growths implanted directly on the left atrial walls or extending into the left atrium through pulmonary veins [11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30].…”

Primary Cardiac Angiosarcoma of Left Atrium

“…In a literature review through PUBMED from 1980 to 2008, more than 250 cases of cardiac angiosarcoma were found; the tumors mostly arose from the right atrium with different extensions and clinical presentations. In the last two decades, only eight cases of this malignant tumor originating from LA were reported in the English literature 1‐8 . This present report describes the ninth case of primary cardiac angiosarcoma arising from an extremely unusual location.…”

Primary left atrial angiosarcoma mimicking severe mitral valve stenosis

“…8 However, we are the first to describe a severe functional mitral valve obstruction caused by a cardiac angiosarcoma. In this case, echocardiography was extremely helpful in describing the morphology, size, and origin of the cardiac mass ( fig 1).…”