Angiosarcoma of the heart. Unusual presentation and survival after treatment.

A 49 year old man presented with severe cyanosis and dyspnoea on exercise. Clinical examination together with echocardiography, cardiac catheterisation, and angiography showed a balloting tumour in the right atrium, intermittently occluding the tricuspid ostium, and an atrial right to left shunt. At operation a pedunculated vascular tumour was found with a broad base which was embedded in the atrial wall and continued into the interventricular septum. Histological examination showed angiosarcomatous features and signs of a less than radical excision. The patient, who made an uneventful recovery, was given postoperative radiotherapy. After 36 months there are no signs of recurrence or metastasis.     

Right atrial hemangioma presenting as pericardial tamponade. A case report

Intracardiac haemangioma is a very rare benign primary cardiac tumour. A 40-year-old patient, with no notable history, presented with chest pain, accentuated by deep inspiration, associated with NYHA stage III dyspnoea. Physical examination revealed signs of right heart failure and electrocardiogram showed low voltage with diffuse repolarization disorders. Chez x-ray showed a cardiothoracic index CTI > 0.70, and enlarged right margins. A large pericardial effusion with a tumour mass filling the right atrial cavity was discovered on transthoracic echocardiography. Chest computed tomography demonstrated the tissue origin of the tumour and suggested extension to the inferior vena cava and hepatic veins. The patient was referred to a surgeon for tumour resection. Histological examination revealed intracardiac capillary haemangioma. Five month postoperative follow-up did not reveal any abnormalities.     

Right atrial spleen.

A large cardiac tumour occupying most of the right atrium and the right ventricle and causing inflow obstruction to the right heart was confirmed by cross sectional echocardiography in a 41 year old man. After surgical resection histological examination showed that the atrial tumour had the characteristics of splenic tissue. Possible mechanisms for the development of such a tumour include an origin analogous to that of an accessory spleen or the implantation and subsequent growth of lymphoid tissue in a pre-existing superior vena caval or high right atrial angioma.     

Right atrial spleen

A large cardiac tumour occupying most of the right atrium and the right ventricle and causing inflow obstruction to the right heart was confirmed by cross sectional echocardiography in a 41 year old man. After surgical resection histological examination showed that the atrial tumour had the characteristics of splenic tissue. Possible mechanisms for the development of such a tumour include an origin analogous to that of an accessory spleen or the implantation and subsequent growth of lymphoid tissue in a pre-existing superior vena caval or high right atrial angioma.     

Right-left shunt caused by sarcoma of the right atrium

A 70 year old woman was admitted for right ventricular failure and cyanosis of recent onset. Echocardiography showed a very large, homogenous, immobile, smooth-contoured mass filling the right atrium. Right atrial pressures were raised but the other intracardiac pressures were normal at catheterisation. Right heart angiography confirmed the voluminous right atrial mass and dilatation of the hepatic veins and showed early opacification of the left heart chambers. A right-to-left shunt was confirmed by oximetry which showed significant desaturation of the blood in the left atrium and ventricle (saturation 78% in the left ventricle). The tumour was also documented by a thoracic CT scan. At surgery, a very large, malignant right atrial tumour was resected which histological examination showed to be an angiosarcoma. The interatrial septum seemed to be intact: there was no true atrial septal defect but a persistent foramen ovale was found. After resection of the tumour the right atrium was reconstructed. The initial postoperative period was uncomplicated but the patient died nine months later of metastatic disease particularly affecting the liver and brain. The clinical presentation of malignant cardiac tumour is very variable but a right-to-left interatrial shunt through a patent foramen ovale has only been reported previously in 2 cases of primary malignant tumours (a rhabdomyosarcoma and an angiosarcoma) and in 1 case of a secondary cardiac metastasis.     

Haemangioma of the right atrium revealed by cardiogenic shock

Intracardiac haemangioma is a very rare primary benign tumour. A 20 year old female patient, with no significant previous medical history, presented to the emergency department with cardiovascular collapse and vague abdominal pains, with no peripheral signs of cardiac failure. The electrocardiogram showed sinus rhythm with diffuse reploarisation disturbances. Chest radiography revealed cardiomegaly (cardiothoracic index of 0.67) with a right paracardial opacity. Abdominal ultrasound showed a moderate peritoneal effusion and transthoracic ultrasound showed a tumour occupying the right atrial cavity but sparing the interatrial septum. The patient underwent emergency open heart surgery for tumour resection and right atrial wall repair with autologous pericardium. Histology confirmed a haemangioma. Follow-up at one month was uneventful. The clinical, diagnostic and therapeutic features of this case are underlined.     

Clinical presentation and non-invasive diagnosis of right heart masses.

Right-sided cardiac masses do not have a uniform clinical presentation. Successful diagnosis is dependent on a high index of suspicion and appropriate echocardiographic examination. Five cases of right sided intracardiac masses have been detected during the period that echocardiography has been routinely available to us--two of right atrial myxoma, one of right atrial thrombus, and two of right ventricular tumour. M-mode echocardiography identified four of the five cases. Two-dimensional echocardiography was necessary to establish the non-invasive diagnosis in the fifth case. Echocardiography should be used in any patient in whom a right sided mass is suspected, or in those patients presenting with signs of tricuspid regurgitation, tricuspid stenosis, pulmonary stenosis, cyanotic heart disease, progressive right heart failure, constrictive pericarditis, or pulmonary emboli without obvious source.     

Primary cardiac angiosarcoma with right coronary-to-right atrium fistula

Primary tumours of the heart are rare. About 25% of all cardiac tumours are malignant and the most common of these is the angiosarcoma. We present a 61-year-old male with a right atrial angiosarcoma that was detected on coronary angiography. The tumour showed marked vascularity and a right coronary-to-right atrium fistula, and the patient underwent surgical resection. Pathological examination of the tumour was consistent with a cardiac angiosacoma and the diagnosis was also confirmed by immuno-histochemistry. He consequently underwent chemotherapy, however the patient died 60 days after the surgery.     

Atrial myxoma. Changes in clinical and paraclinical data. Apropos of 17 cases

The clinical data and presenting signs of 17 cases of atrial myxoma (14 left atrial, 3 right atrial myxomas) were analysed. The aim of the study was to assess changes in the presenting features since the introduction of non-invasive methods of cardiovascular investigation. Two groups of patients were identified according to whether the tumour has been diagnosed before (Group A) or after (Group B) the advent of echocardiography. The diagnosis of cardiac tumour, especially myxoma, has become easier with echocardiography and radio-isotope techniques. The presentation of myxoma, recognised earlier, has changed; the features of advanced valvular disease with resistant heart failure are no longer seen. Variable, atypical clinical signs are now encountered (syncope, pyrexia of uknknown origin, transient ischaemic attacks). The average delay between the first sign and diagnosis was 30 +/- 32 months in Group A, compared to 4,6 +/- 6,5 months in Group B (p < 0, 005). Of the 7 patients in Group B, three had normal cardiac auscultation, and normal ECG, and four had normal ESRs. The diagnosis of myxoma should be considered at the least doubt and an echocardiographic examination, preferably with 2-dimensional echo should be requested. It not only allows positive diagnosis but also orientates the patient to surgery without further investigation.     

Cardiac granulocytic sarcoma diagnosed by intracardiac echocardiography-guided biopsy

A 52-year-old man presented with clinical and echocardiographic signs of cardiac tamponade. A transthoracic echocardiogram revealed a large right atrial mass that obstructed the superior vena cava flow. Cardiac magnetic resonance imaging and computed tomography demonstrated extracardiac tumour invasion of the free atrial wall extending to the right pulmonary hilus. Intracardiac echocardiography-guided biopsy of the tumour revealed the tissue diagnosis-granulocytic sarcoma of the heart. The patient was effectively treated with radiotherapy, chemotherapy and allogeneic haematopoietic stem cell transplantation. He has remained free of the disease for 12 months after treatment.     

Hemangio-endotheliosarcoma of the right atrium

In reporting a case of haemangiosarcoma of the right atrium the authors emphasize the problems encountered in the diagnosis of primary tumours of the heart. The patient was a 50-year old woman who complained of digestive disorders and palpitations. Physical examination showed signs of right heart failure associated with a systolic murmur on the right side of the sternum. ECG gave normal results. Echocardiography displayed a pouch with liquid ultrastructure communicating with the right atrium. Angiography confirmed the presence of a cavity with irregular borders communicating with the right atrium. Coronary arteriography showed an abnormal disorderly distribution of the right coronary artery branches with newly formed vessels extending toward the tumour. At exploratory thoracotomy a large liquid tumour was found which bled at the slightest touch and was attached to the mediastinum and the right pericardium. The pleura and the lung contained several nodules of the same venous colour as the mother tumour. Extemporaneous biopsy and pathological analysis were in favour of a haemangiosarcoma. The patient died a few days after the thoracotomy.     

Atrial septal hematogenous cyst with calcification mimicking myxoma: A rare case

A 61-year-old female presented with right atrial mass during physical examination. Contrast-enhanced left heart echocardiography revealed a mass with the size of 32*23 mm in the right atrium, attached to the atrial septum; there was a certain degree of activity and deformation. MRI showed a mass of about 35*22 mm in the right atrium adjacent to the atrial septum, which was diagnosed with right atrial myxoma. Intraoperative TEE showed that the mass was located in the atrial septum close to the inferior vena cava and spontaneous echo contrast with hyperechoic images within the mass. The lesion was resected under cardiopulmonary bypass. Pathological examination revealed that the filling defect was an atrial septal hematogenous cyst with calcification.     

Massive Left Atrial Sarcoma Presenting With Severe Congestive Heart Failure

Primary intimal sarcomas of the left atrium are extremely rare and highly aggressive tumours. To our knowledge, only 4 cases have been reported to date. We report on a 42-year-old man who presented with signs of congestive heart failure and was found to have an enormous left atrial bilobed tumour with obstruction of the mitral valve. The patient underwent a surgical excision of the sarcoma. The tumour recurred 8.5 months later and the patient required right pneumonectomy.     

Leiomyosarcoma of the left atrium: case report and review of the literature

Leiomyosarcomas are extremely rare primary cardiac tumours. A 46-year-old woman presenting with symptoms and signs of rapidly progressive left ventricular failure and apparent systemic lupus erythematosus was subsequently found to have a grade III/III left atrial leiomyosarcoma which was confirmed surgically. Pathology showed a cellular neoplasm arranged in fascicles with multinucleated giant cells, with areas of high grade sarcomatous change. The patient died seven months postoperatively with intractable heart failure. At autopsy, tumour infiltrated the pericardium, both atria and the right ventricle, with invasion of the diaphragm and posterior mediastinum. The current world literature is reviewed with respect to this rare and often misdiagnosed tumour.     

Recurring cardiac myxoma.

Of a series of 14 patients surviving operation for atrial myxoma, two developed signs of recurrence of the tumour. In both cases the patients underwent two further separate operations for recurrent lesions. The time before the second recurrence was nearly 11 years and four and a half years. In neither case did histological examination show malignant change. These two cases of recurrent atrial myxoma, together with four other reported cases, indicate that a second recurrence may occur in about 25% of patients with a first recurrence. Multiple foci of tumour growth is probably the explanation for recurrence in most cases.     

Kidney tumour mimicking cardiac mass

The discovery of a mass in the right atrium obliges the clinician to perform a broad differential diagnosis between a primary cardiac tumour (with myxoma being the most frequent), invasion of an extracardiac tumour, vegetations on the tricuspid valve, and an atrial thrombus. We describe the case of a patient who was admitted to our service with a diagnosis of suspected myxoma based on the chance transthoracic echocardiographic discovery of a right atrial mass. A transesophageal echocardiogram showed the process to be extracardiac, and magnetic resonance imaging showed it to originate at the renal level extending via the inferior vena cava to the right atrium. Tumour extension with thrombosis of the vena cava is a relatively frequent complication of renal carcinoma, but only exceptionally does it reach the right atrium. It is also exceptional that this was a chance finding in an asymptomatic patient.     

Recurring cardiac myxoma

Of a series of 14 patients surviving operation for atrial myxoma, two developed signs of recurrence of the tumour. In both cases the patients underwent two further separate operations for recurrent lesions. The time before the second recurrence was nearly 11 years and four and a half years. In neither case did histological examination show malignant change. These two cases of recurrent atrial myxoma, together with four other reported cases, indicate that a second recurrence may occur in about 25% of patients with a first recurrence. Multiple foci of tumour growth is probably the explanation for recurrence in most cases.     

Right atrial tumour and right ventricular failure - diagnostic role of echocardiography and cardiovascular magnetic resonance

A case of a 66 year-old female with advanced right ventricular failure is described. Echocardiography and MRI revealed the presence of right atrial tumour. The patient underwent successful surgery and histological examination revealed lymphoma.     

Myxoid leiomyosarcoma of the left atrium: a rare malignancy of the heart and its comparison with atrial myxoma

Primary tumours of the heart are rare. The majority of these tumours are benign, with myxomas located in the left atrium being the most common form. Almost all malignant tumours are sarcomas and occur preferentially in the right side of the heart. An exception to this rule is leiomyosarcoma, a rare form of primary cardiac sarcoma that occurs predominantly in the left atrium, as does cardiac myxoma. The case of a 53-year-old woman who presented with symptoms of mitral valve stenosis and pulmonary hypertension is reported. Cardiac catheterization, angiography and echocardiography revealed a left atrial mass that was interpreted as atrial myxoma. At the time of operation, the myxoid appearance of the tumour mass further supported this assumption. The tumour, including a wide rim of atrial septum, was removed with cautery. Histopathological examination unexpectedly showed that the tumour was not an atrial myxoma but rather a myxoid variant of a primary leiomyosarcoma. Immunohistochemistry and electron microscopy confirmed the diagnosis. Local radiotherapy was considered but deemed contraindicated in view of the longstanding pulmonary hypertension. Two months after excision, a repeat echocardiogram indicated recurrence of tumour in the left atrium, and the patient died a few days later. The preferential left atrial location and the frequently myxoid appearance of primary leiomyosarcomas of the heart make it particularly difficult to differentiate them preoperatively from atrial myxomas. The authors recommend resection of all atrial myxoid tumours with a wide (at least 1 cm) margin, combined with intraoperative frozen section diagnosis, because complete surgical resection appears to correlate with prolonged survival in the few reported cases of atrial leiomyosarcomas. In cases of incomplete initial resection or local recurrence in the absence of metastatic disease, heart transplantation may be a valid option in appropriately selected patients.     

Congenital mitral and tricuspid double stenosis

The authors report the case of a child followed up from the age of 4 months to the age of 15 for asymptomatic congenital heart disease. Initial clinical examination showed the presence of a systolic murmur and a diastolic rumble in the xiphoid area with signs of right atrial dilatation-hypertrophy. During follow-up, an apical diastolic rumble was detected with left atrial dilatation from the age of 3. Doppler echocardiography carried out recently showed double mitral and tricuspid valve stenosis with two-dimensional appearances of doming of both valves and moderate transvalvular pressure gradients with slight elevation of pulmonary artery pressures on the Doppler study. The very early detection of the malformation and the continuous follow-up of this patient indicate the congenital and not rheumatic origin of this double valve stenosis.