A case of primary cardiac lymphoma located in the pericardial effusion

Primary cardiac lymphoma is a rare disorder with a poor prognosis. We present here a case of 77-year-old woman who was diagnosed as having cardiac lymphoma antemortem according to a cytologic examination of the pericardial effusion. Determination of the levels of serum-soluble interleukin-2 receptor and serum deoxythymidine kinase was useful for the diagnosis. Echocardiography, computed tomography, magnetic resonance imaging, and gallium scan revealed neither lymphadenopathy nor tumor in the heart, so she was diagnosed as having malignant lymphoma that probably originated from the pericardium. Systemic chemotherapy with CHOP (cyclophosphamide, farmorubicin, oncovin, and prednisolone) resulted in a complete resolution of the pericardial effusion. She has been in remission 48 months after discontinuation of the chemotherapy.     

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.     

Primary cardiac lymphoma: diagnosis by transjugular biopsy

Primary cardiac lymphoma, defined as a non-Hodgkin's lymphoma involving only the heart and pericardium, is an extremely rare malignancy. It should be suspected in patients with a heart mass and heart failure, unexplained refractory pericardial effusion or rhythm disturbances. Transvenous intracardiac tumor biopsy under fluoroscopic or transesophageal echocardiographic guidance, is a minimally invasive technique which makes definite diagnosis possible. We describe a patient in whom primary cardiac lymphoma was diagnosed by this technique. He also underwent percutaneous balloon pericardiotomy because of severe refractory pericardial effusion. Seven months after diagnosis and treatment with standard chemotherapy, the patient remained free of disease.     

Primary effusion lymphoma complicating cardiac tamponade: a case report

A 76-year-old woman was admitted to our hospital because of exertional dyspnea and leg edema during the previous month. Her systolic blood pressure on admission was 80 mmHg with 12 mmHg of pulsus paradoxous, and her pulse rate was 110 beats/min. Chest radiography revealed marked cardiomegaly and echocardiography showed massive pericardial effusion mainly behind the left ventricle and collapse of the right ventricle. The initial diagnosis was pericardial tamponade. Pericardiocentesis and pericardial drainage revealed bloody pericardial effusion. After drainage, her vital signs improved and her symptoms immediately disappeared. The cytological analysis of the pericardial effusion revealed numerous lymphoma cells. Computed tomography of the neck, chest and abdomen showed no evidence of tumor masses, lymph node enlargement, or hepatosplenomegaly. Infectious disease, collagen disease and aortic dissection were excluded. The final diagnosis was primary effusion lymphoma. The prognosis of primary effusion lymphoma is generally unfavorable because it is frequently accompanied by immunodeficiency disease. However, there was no human immunodeficiency virus infection in this patient. Fortunately, the effect of chemotherapy was excellent and the patient is doing well 1 year after the diagnosis.     

“Bronchogenic cyst impersonating a pericardial lesion”: A case report

A 32-year-old female presented with palpitations and chest discomfort. The patient had a history of pericardiotomy due to pericardial effusion. Multimodal imaging, including echocardiography, cardiac magnetic resonance (CMR), and coronary computed tomography angiography (CCTA) showed a single mass in the pericardium as the cause of the symptoms. Furthermore, its location and potential complications were accurately defined. The patient underwent a successful surgical resection of the pericardial cyst, microscopic histopathological examination was compatible with a bronchogenic cyst, a very rare congenital malformation. The article discusses the rarity of bronchogenic cysts in the pericardium and the importance of accurate diagnosis and appropriate treatment.     

Transesophageal echocardiographic observation of multiple myeloma involving the pericardium: a case report

This report presents a case of multiple myeloma with a mass lesion on the pericardium and pericardial effusion. The response to intrapericardial combination chemotherapy (cisplatin and betamethasone) was evaluated by repeated transesophageal echocardiograms. Following the treatment, complete resolution of the tumor and effusion were observed for 6 months after which the patient died of bacterial pneumonia. Intrapericardial combination chemotherapy can be an effective treatment for myelomatous involvement of the pericardium.     

Primary malignant pericardial mesothelioma temporarily reduced by radiation therapy: a case report

A 47-year-old woman was referred to our hospital because of cardiomegaly and pericardial effusion. She complained of a cough. Computed tomography, echocardiography, and magnetic resonance imaging showed a mass on the pericardium. Exploratory surgery revealed a solid tumor invading the pericardium over the aortic arch and main pulmonary artery. Histological examination indicated primary malignant pericardial mesothelioma. After 58 Gy radiation, the size of the tumor was temporarily reduced and the patient's symptoms disappeared. However, the tumor enlarged and her symptoms reappeared 7 months after temporary improvement. Eighteen months after the development of cough, the patient died suddenly.     

Malignant lymphoma diagnosed by the pericardial biopsy

The biopsy findings of malignant lymphoma involving the pericardium in a 69-year-old male were reported. The initial presenting symptoms were heart failure followed by rapidly progressive complete heart block. Pacemaker was inserted. Chest roentgenogram showed a marked left pleural effusion. An echocardiogram demonstrated pericardial fluid. The diagnosis of lymphoma was suggested by gallium isotope study with markedly increased isotope uptake in heart, and was subsequently confirmed by pericardial biopsy. After chemotherapy the pleural effusion was decreased and the uptake of gallium disappeared. While the diagnosis of cardiac involved lymphoma was hard antemortem, experience in this case suggests that gallium isotope study and pericardial biopsy are helpful in establishing the correct diagnosis.     

Primary high-grade myofibroblastic sarcoma arising from the pericardium.

Primary pericardial sarcomas are very rare. A 62-year-old Japanese man presented with cardiac tamponade. Echocardiography, computed tomography and magnetic resonance imaging revealed massive pericardial effusion and a large tumor in the pericardial cavity, attached to the pericardium of the left ventricular posterolateral free wall. Surgical excision of the tumor was performed and histopathological and immunohistochemical examinations identified high-grade myofibroblastic sarcoma. Because of local recurrence soon after surgery, the patient received adjuvant chemotherapy, including doxorubicin and ifosfamide, and subsequent radiotherapy. As of 6 months after completing radiotherapy, the patient was alive and no disease progression or distant metastases were evident. This may be the first report of primary high-grade myofibroblastic sarcoma arising from the pericardium.     

Two cases of primary cardiac lymphoma presenting with pericardial effusion and cardiac tamponade

The heart as the primary site of lesion in malignant lymphoma is extremely rare. We experienced 2 cases of malignant lymphoma whose initial presentation was massive pericardial effusion with cardiac tamponade. The first case was a 75-year-old man who had shortness of breath for 1 week. Chest X-ray showed cardiomegaly (CTR 65%), and his condition was diagnosed as congestive heart failure at first and thereafter echocardiogram revealed pericardial effusion. The second case was a 76-year-old man who complained of exertional dyspnea which worsened over 2 weeks. His condition was diagnosed as congestive heart failure at first and echocardiogram revealed pericardial effusion. Pericardial drainage tapped bloody fluid and cytological examination revealed malignant lymphoma. After treatment, the first case lived for eleven months with no recurrence of pericardial effusion. The second case has lived for 4 years. We present these 2 cases can be defined as primary cardiac lymphoma according to McALLISTER and FENOGLIO, i.e., a lymphoma that involves only the heart and pericardium. More cases of primary cardiac lymphoma will be found in the future because of the ease with which the echocardiogram can detect pericardial effusion.     

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.     

Cardiac tamponade as the first symptom of oesophageal carcinoma -- a case report

We present a case of a 56-year-old male who was admitted to our hospital due to cardiac tamponade. A 600 ml of purulent fluid was evacuated from the pericardium. The patient received antibiotics, however, due to recurrent pericardial effusion a pericardial drainage was required. Esophagoscopy and computerised tomography revealed oesophageal carcinoma with oesophago-pericardial fistula.     

Carcinomatous pericarditis monitored by pulsed Doppler echocardiography of superior vena cava: hemodynamics from pericardial effusion to pericardial solid invasion]

We utilized pulsed Doppler echocardiography to monitor changes in a patient with carcinomatous pericarditis, from pericardial effusion with cardiac tamponade to pericardial solid invasion. Characteristic changes in superior vena cava (SVC) pulsed Doppler flow patterns were observed after invasion of the pericardium. SVC pulsed Doppler flow patterns are a useful aid in differentiating between solid invasions and recurrent pericardial effusion with pericardial thickening: this diagnostic capability is not readily available with computed tomography.     

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.     

Magnetic resonance imaging of constrictive pericardial disease

Gated magnetic resonance imaging of 5 patients with suspected constrictive pericardial disease was performed using a superconducting magnet operating at 0.35 Tesla. Results were compared with those of echocardiography and hemodynamic measurements in all patients, with chest films in 5, computerized tomography in 2 and with histologic findings in 3. Pericardial thickness exceeded 5 mm in 4 patients and was 5 mm in 1 patient. Absence of magnetic resonance signal from the thickened pericardium was observed with extensive calcific deposits, and increased intensity of the thickened pericardium was associated with inflammatory disease. Dilatation of the right atrium, venae cavae and hepatic veins, and right ventricular narrowing was observed in all patients. The ventricular septum was straight in all patients. Magnetic resonance imaging allows both measurement of pericardial thickness and depicts internal cardiac anatomy without exposure to radiation or use of contrast medium. Satisfactory imaging with a large field of view can be performed in the presence of lung disease, thoracic deformity or surgical "hardware"--conditions that limit echocardiography and computerized tomography. The inherently 3-dimensional data permit imaging in any plane without loss of resolution. Thus, magnetic resonance appears to be the noninvasive method of choice for the diagnosis of constrictive pericardial disease.     

Enlarged left atrium causing possible false positive echocardiographic diagnosis of pericardial effusion.

Echocardiographic examination in a patient with IHSS, mitral insufficiency, and cardiomegaly suggested the possibility of a pericardial effusion. Echocardiographic M-mode scanning documented the continuity of the space posterior to the pericardium with an enlarged left atrium. Subsequent cineangiographic studies confirmed an enlarged left atrium and the absence of pericardial effusion. Left atrial enlargement may cause a false positive echocardiographic diagnosis of pericardial effusion.     

Constrictive pericarditis caused by a pericardial-occupying tumor due to esophageal cancer

A 61-year-old female was admitted to our hospital for esophageal cancer treatment. Esophagectomy with 2-field lymphadenectomy was performed. Postoperative findings revealed the lesion was a poorly differentiated squamous cell carcinoma invading into the diaphragm and there were no carcinoma cells on the surgical margins. Eight months after surgery, a recurrence was suspected by the presence of tumors at the pericardia, right axillary lymph node and around the descending aorta. The patient was re-admitted for chemotherapy and administrated fluorouracil and cisplatin 4 days after admission. After 7 days, she complained of dysphagia. Esophagogastroduodenoscopy showed no abnormal lesion that could cause the symptom. Computed tomography revealed massive progression of the pericardial tumor, bilateral pleural effusion and congested liver. Echocardiography showed the diffuse pericardial tumor caused restriction of ventricular dilation and hemodynamics of constrictive pericarditis. The patient died 29 days after re-admission. Autopsy revealed squamous cell carcinoma involving the mediatinum and pericardium. The pericardium was completely full of cancer tissue but no fluid. We concluded that the direct cause of death was neoplastic constrictive pericarditis.     

Durable remission by sobuzoxane in an hiv-seronegative patient with human herpesvirus 8-negative primary effusion lymphoma

Primary effusion lymphoma (PEL) is recognized as a unique clinicopathological entity associated with human herpesvirus 8 (HHV-8), and it occurs almost exclusively in human immunodeficiency virus (HIV)-infected individuals. In the majority of PEL cases, Epstein-Barr virus (EBV) has been found in the tumor cells as well. We describe here an elderly HIV seronegative female patient with PEL in the pleura and pericardium not associated with HHV-8 or EBV. Cytologic examinations of the pleural effusion revealed large lymphoma cells with immunophenotypes positive for CD8, CD10, CD19, CD20, CD22, CD24, CD45, and HLA-DR but negative for CD30 and surface immunoglobulin. Chromosome analysis showed complicated abnormalities including add(3)(q27). Immunoglobulin gene rearrangement was detected by Southern blotting; however, c-myc, Bcl-2, and Bcl-6 genes were not rearranged. The patient was treated with a modified CHOP (cyclophosphamide, hydroxydoxorubicin, oncovine, and prednisolone) regimen, and achieved remission. Recurrence of PEL in the pericardium as effusion lymphoma was found 3 months after the discontinuation of CHOP. After approximately 1 year of intermittent multiagent salvage therapy for pericardial recurrences, a treatment that resulted in a partial response, 3 cycles of monotherapy with sobuzoxane were administered. At the time of this report the patient had been free from PEL for more than 18 months without chemotherapy.     

Efficacy of rituximab monotherapy for an elderly hemodialysis patient with primary cardiac lymphoma

We report a case of primary cardiac lymphoma (PCL) occurring in a 76-year-old man during maintenance hemodialysis. Chest computed tomography (CT) revealed a tumor with pericardial effusion in the left ventricular posterior wall. Cytological examination of the pericardial fluid revealed monotonous lymphoid cells positive for B-cell markers, and clonal immunoglobulin heavy chain gene rearrangement was detected, indicating B-cell lymphoma. Rituximab monotherapy was administered biweekly at the therapeutic level on hemodialysis. The follow-up chest CT showed tumor disappearance with pericardial fluid after two courses of therapy. Rituximab monotherapy was effective for an elderly hemodialysis patient with PCL.     

A young man with intractable ascites and effort dyspnoea without echocardiographic signs of pericardial thickening: the importance of clinical investigation, CT scan and MRI in the diagnosis of constrictive pericarditis

A 35-year-old male patient suffering from dyspnoea on effort for 8 months, with abdominal and jugular venous distension, was previously studied in another hospital and discharged with a diagnosis of restrictive cardiomyopathy. Physical examination revealed a blood pressure of 110/60 mm Hg and absence of pericardial knock and also of paradoxical pulse. Chest X-ray showed no cardio-pulmonary alterations. Transthoracic echocardiography showed mild LV dysfunction (LVEF 46%) and lack of pericardial effusion and thickening. Doppler interrogation of transmitral flow showed a restrictive pattern. Computed tomography showed diffusely thickened pericardium, with the absence of calcification and of pericardial effusion. Cardiac magnetic resonance confirmed pericardial thickening and showed lack of myocardial alterations. Mild LV dysfunction was noted with dyskinesia of interventricular septum. The patient underwent cardiac catheterization, demonstrating an equalisation of RV and LV diastolic pressures with “square root” sign. The patient underwent pericardiectomy with consequent resolution of his symptoms and improvement of LV function.