Primary cardiac lymphoma causing right atrial occlusion

Primary cardiac lymphomas (PCL) are extremely rare, and diffuse large B-cell lymphoma is a highly aggressive subtype. We report a case that was initially diagnosed as chronic right heart dysfunction. Detailed investigations revealed a large lobulated tumour occluding the right atrium, infiltrating the inter-atrial septum, the roof of the left atrium, and the aortic root. Despite adequate surgical debulking and initial successful tricuspid annuloplasty, the patient succumbed to multi-organ failure. Pathological analysis of the resected tumour confirmed a diffuse large B-cell lymphoma with a proliferation rate of 100%. What is unique about this case is the size of this rare cardiac tumour, which we believe to be one of the largest described in the literature for a purely intra-cardiac PCL, its aggressive growth rate, and the relatively mild symptomatology until a late stage of the disease.     

Successful treatment of two patients with primary cardiac malignant lymphoma

We describe two patients with primary cardiac malignant lymphoma involving the right atrium and superior vena cava, resulting in intractable right cardiac failure and superior vena cava syndrome. Patients were diagnosed by surgical myocardial biopsy and were treated with combination chemotherapy for non-Hodgkin's lymphoma. Each attained a marked response, and hence avoided sudden death from tricuspid atresia. Both have remained alive for more than 21 and 34 months, respectively, and continue intermittent combination chemotherapy.     

Primary cardiac lymphoma. Report of a case

Malignant non-Hodgkins lymphomas have a secondary cardiac localisation in 20% of cases. However, a cardiac primary site is rare (44 cases described up to now). A positive diagnosis is rarely made before death. There is great interest in echocardiography, a non-invasive method, to identify these tumours early. The prognosis remains nevertheless gloomy. We report the case of a child aged 8 years, admitted with a scenario of low output right cardiac insufficiency. Chest radiography identified cardiomegaly with a prominent right border, and the electrocardiograph showed right auricular hypertrophy. A tumour mass infiltrating the right atrium, the right ventricle and the lateral face of the left ventricle was discovered on trans-thoracic echocardiography. Investigation for tumour spread was negative. The patient died before operation in a state of extreme low output. The histology favoured a highly malignant non-Hodgkins lymphoma type B.     

Primary Cardiac Lymphoma: Case Report and Brief Review of the Literature

Primary cardiac lymphoma is defined as a non-Hodgkin's lymphoma mainly located in the heart and/or the pericardium. It is rare and affects elderly men. Common manifestations are pericardial effusion and heart failure. Diagnosis is usually late and prognosis is poor. We report a case of a patient with a large primary cardiac lymphoma who presented with chest pain and negative T-waves in electrocardiogram. Transthoracic echocardiogram showed a large mass in the right atrium and right ventricle while transesophageal echocardiogram also revealed the presence of large mobile masses in the right atrium, which were considered to be thrombi. Tissue biopsy showed a high-grade B-cell diffuse lymphocytic lymphoma. The patient was treated with chemotherapy and radiation with complete remission and prolonged survival.     

Primary cardiac lymphoma: diagnosis and treatment. Report of 6 cases and review of the literature

Primary cardiac lymphoma (PCL) is the rarest primary cardiac tumour and carries a poor prognosis. Early diagnosis, often difficult, to introduce appropriate treatment as soon as possible, seems to have a positive impact on prognosis. The authors report their experience of 6 patients with PCL. None of the patients had immune depression. The presentations were tamponade (N= 2), right heart failure (N= 1), general ill health (N= 3). A PCL was suspected on echocardiography and thoracic CT scan showing tumour invading the right heart chambers in all cases. The diagnosis of PCL was confirmed by surgical biopsy in 5 patients and by endomyocardial biopsy in 1 patient. A diffuse large cell type B lymphoma was found in 5 patients and an anaplastic lymphoma in 1 patient. One patient died of right heart failure 4 days after diagnosis and before starting chemotherapy. All the other patients received chemotherapy. Two patients died during their first course. The other three patients had several courses of chemotherapy: there are two survivors 17, 5 months later and one patient died 62 months after diagnosis. The diagnosis of PCL should be suspected in patients with a cardiac tumour associated or not with pericardial effusion. Early, appropriate chemotherapy seems to have a positive impact on the prognosis, justifying aggressive approaches to obtain a rapid histological diagnosis.     

Primary cardiac lymphoma presenting clinically as restrictive cardiomyopathy.

An unusual case of primary cardiac lymphoma presenting as restrictive cardiomyopathy with arrhythmia is reported in a 72-year-old woman who was admitted for evaluation of exertional dyspnea and palpitations. Electrocardiography (ECG) showed atrioventricular dissociation and right heart cardiac catheterization revealed a typical 'dip-and-plateau' waveform. Restrictive cardiomyopathy was suspected because computed tomography (CT) did not reveal pericardial thickening, calcifications, or an effusion. Heart failure initially improved with diuretic therapy, but subsequently worsened, and the patient experienced a syncopal episode. ECG showed atrial fibrillation, and CT revealed a large mass in the right atrium and multiple tumors in the liver, which needle biopsy confirmed as diffuse large B-cell lymphoma. Chemotherapy induced complete remission, and her heart failure markedly improved. The 'dip-and-plateau' waveform was no longer detected on repeat cardiac catheterization and the ECG showed restoration of sinus rhythm. Clinically, the diagnosis was primary cardiac lymphoma.     

Synovial sarcoma in the right atrium and right ventricle

A metastatic synovial sarcoma in the right atrium and ventricle is described. A 36-year-old man was admitted to our hospital with generalized fatigue, dyspnoea, and precordial pain. Transthoracic echocardiography demonstrated a metastatic tumour in both the right atrium and right ventricle and revealed obstruction of the inflow tract of the right ventricle caused by a metastatic right atrial tumour. Thoracic computed tomography revealed a pleural-based paravertebral mass in the left intrathoracic cavity and multiple pulmonary nodules in both lungs. Cardiac surgery was performed for palliative treatment due to right cardiac failure and a risk of fatal embolization. The patient died 12 months after the cardiac surgery.     

Diagnosis of cardiac tumor by percutaneous thromboaspiration. A case report

Percutaneous endovascular thromboaspiration is a valuable tool as illustrated by the case of a patient suffering from a large intracardiac tumour. Histological and immuno-histochemical analysis of the tumour fragments provided the diagnosis of a cardiac angiosarcoma. The patient was a 44 year old man admitted for a large sero-sanguinous pericardial effusion which recurred after drainage. The case was complicated by a haemorrhagic cerebrovascular accident unrelated to a secondary deposit. Initially suspected after transthoracic echocardiography, the diagnosis of a tumour invading the right atrium was confirmed by transoesophageal echocardiography and cardiac CT scan. Surgery was declined and as the diagnosis of lymphoma could not be excluded, the patient underwent biopsy by an original method of percutaneous thromboaspiration. This minimally invasive, low cost technique would appear to be a valuable alternative to other endovascular biopsy techniques (saber, biotome) and to surgical biopsy, and could be proposed as the technique of first intent in an a priori non-operable intra-cardiac tumour or when lymphoma is suspected.     

Primary cardiac lymphoma--a case report

Primary cardiac lymphoma, which is very rare, is generally regarded to have a poor prognosis. A case of a 69-year-old man with primary cardiac lymphoma diagnosed by antemortem examination is reported. A computed tomography scan of the chest demonstrated a huge right atrial mass with invasion into the other chambers. No mediastinal lymphadenopathy was detected. Cytologic analysis of pericardial effusion revealed diffuse large B-cell type non-Hodgkin malignant lymphoma. The patient died on the 18th day of chemotherapy (cyclophosphamide, hydroxydaunomycin, oncovin, and prednisone) due to low-output syndrome and multiple organ failure. At autopsy, massive gray-white tumor almost occupied the right atrium and invaded the right inferior lobe of the lung. Although prognosis of primary cardiac lymphoma remains poor, early diagnosis may improve the prognosis.     

Severe nonthrombotic pulmonary embolism due to primary cancer of the pancreas--a case report

We describe a patient with a severe, neoplastic pulmonary embolism due to primary cancer of the pancreas. The 40-year-old male was admitted to the haematology department with an initial diagnosis of lymphoma. Transthoracic echocardiography showed a mass in the right atrium. Transoesophageal echocardiography was performed and revealed two moving tumours: the first in the extend of the vena cava superior and the second one in the tricuspid valve annulus. After bronchoscopy the patient's haemodynamic condition deteriorated and was followed by cardiac arrest. Post-mortem examination showed extensive tumour emboli in the left pulmonary artery as well as pancreatic cancer at an advanced stage.     

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.     

Multiple intracavitary cardiac masses; an uncommon presentation of African Burkitt's lymphoma.

A 29-yr-old patient is described, who presented with rapid general deterioration and right heart failure. Two-dimensional echocardiography revealed massive intracardiac tumor involving the right atrium, the right ventricle and the left atrium. At histology the diagnosis of Burkitt's lymphoma was made. This report presents what we believe to be the first case of a patient with primary cardiac manifestations and multiple intracardiac masses in this disease.     

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.     

Primary Cardiac Lymphoma: Echocardiography and F‐18‐Fluorodeoxyglucose Positron Emission Tomography in Selection of a Biopsy Site

A 63‐year‐old man was referred to our hospital because of a cardiac tumor. Transthoracic echocardiography revealed a rough, mobile tumor in the dilated right atrium, and transesophageal echocardiography showed that the tumor consisted of small, botryoidal masses. Catheter‐based biopsy carried a high risk of embolism. Therefore, we used F‐18‐fluorodeoxyglucose positron emission tomography (FDG‐PET), which revealed an abnormal accumulation in the right cervical lymph nodes, as well as in the heart. We safely performed biopsy of the lymph nodes and diagnosed the patient with primary cardiac lymphoma. We concluded that echocardiography and FDG‐PET are useful for selecting an appropriate biopsy site in primary cardiac lymphoma.     

Clinical perspectives of primary cardiac lymphoma

Primary cardiac lymphoma is a very rare malignancy, which is typically of a non-Hodgkin type, and involves only the heart and pericardium with no or minimal evidence of extracardiac involvement. Primary cardiac lymphoma account for about 1% of the primary cardiac tumors and 0.5% of the extranodal lymphomas. On the other hand, disseminated lymphoma with cardiac involvement can occur in up to 20% of patients with lymphoma. About 80% of cases of the primary cardiac lymphoma in immunocompetent hosts are of diffuse B-cell lymphoma, and in patients with immunodeficiency states, small noncleaved or immunoblastic lymphomas are more frequent. The right atrium and right ventricle are the 2 most frequently involved sites. Clinical presentation is heterogeneous and is generally related to the site of involvement in the heart. The diagnosis is suspected when patients present with a cardiac mass or an unexplained refractory pericardial effusion. A thorough workup should include transthoracic and transesophageal echocardiography, computed tomography, and magnetic resonance imaging. Diagnosis is confirmed by cytology of the serous fluid from pericardial or pleural effusion or biopsy of the pericardial mass or endomyocardial tissue. The exploratory thoracotomy should not be delayed if indicated. Chemotherapy has been used alone or combined with radiotherapy. Similarly, palliative cardiac surgery has been performed, mainly for tumor debulking. Combination of chemotherapy and radiation therapy is considered as the treatment of choice. The survival is generally less than a month without treatment but has been prolonged up to 5 years with palliative treatments in selected 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.     

Primary cardiac lymphoma

Primary cardiac lymphoma is a rare entity. We report on the clinicopathological features of 2 patients with primary cardiac lymphomas: one involving the right atrium resulting in intractable right heart failure, and the other involving the pericardium with massive pericardial effusion. In the first patient, sternotomy and surgical biopsy of the tumor were performed to arrive at the diagnosis. In the second patient, CT thorax and transesophageal echocardiography helped to diagnose the pericardial tumor, and cytological examination of the pericardial fluid established the pathological diagnosis of lymphoma. Combination chemotherapy (COPP) was started in both patients. The first patient died on the first day of chemotherapy due to intractable heart failure, while the second attained a partial response to chemotherapy but died of progressive disease 8 weeks later. This is followed by a literature review of 21 patients with primary cardiac lymphoma. In conclusion, the prognosis of primary cardiac tumor remains poor. Am. J. Hematol. 54:79–83, 1997 © 1997 Wiley-Liss, Inc.     

Malignant lymphoma of the heart. Contribution of modern technics of medical imaging

Although rarely encountered, tumours of the heart are now more easily and frequently diagnosed owing to the availability of non-invasive imaging methods, notably echocardiography. With echocardiography the cardiac extension of the tumour is perfectly visualized, but its connections with the mediastinum are less readily accessible. The case presented here of a female patients with a malignant lymphoma involving the heart (right ventricle and atrium, pericardium) and the mediastinum illustrates the complementary nature of echocardiography, computerized tomography and magnetic resonance imaging.     

Cavernous hemangioma of the right atrium

Cardiac hemangioma is a rare primary benign tumour, localised in the right atrium in 23% of cases. In a 60-year-old patient, who complained of remote chest discomfort and recent exertional palpitation, a right atrial mass was discovered by magnetic resonance imaging and echocardiography. A selective operation was performed and the tumour was resected. Histology revealed it to be a cavernous hemangioma. Due to the potential risks associated with cardiac hemangioma, surgical resection and postoperative follow-up are recommended.