Primary B-Cell Lymphoma of the Heart

11/10/2005

Question:  What is the incidence and presentation of primary B-cell lymphomas of the heart?

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<1>

Unique Identifier [PMID]: 15160956

Authors: Anghel G. Zoli V. Petti N. Remotti D. Feccia M. Pino P. Majolino I.

Institution: Hematology and Bone Marrow Transplantation Unit, Ospedale "S. Camillo-Forlanini", Rome, Italy.

Title: Primary cardiac lymphoma: report of two cases occurring in immunocompetent subjects. [Review] [80 refs]

Source: Leukemia & Lymphoma. 45(4):781-8, 2004 Apr.

Abstract: Primary cardiac lymphomas (PCLs), involving solely heart and/or pericardium at presentation, are rare events. They are frequently recognized at autopsy and generally carry a poor prognosis due either to a delay in the diagnosis or to infiltration of heart structures. We report here on two patients with large B-cell PCL. One is a 52-year-old man who presented with multiple cardiac tumors infiltrating mainly the right atrium and the inter-atrial septum. Diagnosis was established by ultrasound-assisted transesophageal biopsy of the intra-atrial multilobated tumor mass. He was treated with Rituximab-implemented high-dose sequential (R-HDS) chemotherapy followed by autologous peripheral blood stem cell transplantation, attaining complete response. He had no evidence of disease 24 months from onset. The second patient was a 70-year-old woman who presented with pericardial tamponade and low-output cardiac failure. Despite prompt pericadiocentesis and chemotherapy with cyclophosphamide and vincristine, she died 2 weeks later. Postmortem examination revealed large B-cell lymphoma proliferation confined to the heart. Whether primitive heart localizations represent an independent prognostic factor, and what specific measures should be adopted in patients with this rare presentation is the subject of the present report and review of the literature. [References: 80]

Publication Type: Case Reports. Journal Article. Review. Review of Reported Cases.

<2>

Unique Identifier [PMID]: 14728061

Authors: Sarjeant JM. Butany J. Cusimano RJ.

Institution: Department of Anaesthesia, University of Toronto, Toronto, Ontario, Canada.

Title: Cancer of the heart: epidemiology and management of primary tumors and metastases. [Review] [62 refs]

Source: American Journal of Cardiovascular Drugs. 3(6):407-21, 2003.

Abstract: Cardiac tumors, benign or malignant, are rare and most are benign. The most common benign tumor is the cardiac myxoma. Malignant cardiac tumors are usually sarcomas. The pericardium can be the site of benign and malignant cardiac tumors, though metastatic tumors occur here far more commonly than do primary tumors. Successful treatment for benign cardiac tumors is usually achieved by surgical resection. Surgery for primary malignant tumors is, however, much less successful as complete resection is usually not possible. Primary cardiac lymphoma may be successfully treated by chemotherapy. Tumors that metastasize to the heart from other organs occur 100- to 1000-fold more commonly than primary cardiac tumors. Metastatic spread to the heart has been identified in approximately one-fifth of all patients who have metastatic cancer with lung carcinoma being the most common primary tumor. Symptoms of cardiac metastases vary, and they depend on the site and extent of the lesions. Treatment varies depending on the pathology of the primary tumor. However, the aim of treatment is usually symptomatic relief. With the advent of AIDS, Kaposi's sarcoma and high grade B cell lymphomas have also been identified in cardiac tissue. The aim of this article is to review the epidemiology, clinical presentation, pathology and treatment of cardiac tumors. [References: 62]

Publication Type: Journal Article. Review. Review, Tutorial.

<3>

Unique Identifier [PMID]: 14565636

Authors: Gowda RM. Khan IA.

Institution: Division of Cardiology, Long Island College Hospital, Brooklyn, NY, USA.

Title: Clinical perspectives of primary cardiac lymphoma. [Review] [62 refs]

Source: Angiology. 54(5):599-604, 2003 Sep-Oct.

Abstract: 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. [References: 62]

Publication Type: Journal Article. Review. Review, Tutorial.

<4>

Unique Identifier [PMID]: 12447675

Authors: Chalabreysse L. Berger F. Loire R. Devouassoux G. Cordier JF. Thivolet-Bejui F.

Institution: Department of Pathology, Hopital Louis Pradel, BP Lyon Montchat 69394 Lyon, Cedex 03, France. lara.chalabreysse@chu-lyon.fr

Title: Primary cardiac lymphoma in immunocompetent patients: a report of three cases and review of the literature. [Review] [42 refs]

Source: Virchows Archiv. 441(5):456-61, 2002 Nov.

Abstract: Primary cardiac lymphoma is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium, extremely rare in immunocompetent patients, and more frequent in immunodepressed patients. We present 3 retrospectives cases of primary cardiac lymphoma in immunocompetent patients and review 35 cases reported in the literature. Two patients were adults and one was a child. Primary cardiac lymphoma presented with constitutional symptoms in two cases and superior vena caval syndrome in one case. Diagnosis of a tumor mass was made in all cases by transthoracic echocardiography. Primary cardiac lymphoma arose in the heart right chambers in two cases. Histological diagnoses, obtained after thoracotomy, were diffuse large B-cell lymphoma in two cases, and Burkitt's lymphoma in one case. All three cases received chemotherapy, combined with radiotherapy in one patient. Of our patients, 2 are alive and asymptomatic 12 months and 33 months after diagnosis. In conclusion, diagnosis of primary cardiac lymphoma is difficult due to non-specific clinical manifestations and should be considered in patients with a cardiac mass sometimes with pericardial effusion. It is confirmed using transthoracic echocardiography and magnetic resonance imaging and certified using cytology or open biopsy. The only effective treatment is chemotherapy, but prognosis remains poor. [References: 42]

Publication Type: Case Reports. Journal Article. Review. Review of Reported Cases.

<5>

Unique Identifier [PMID]: 11734310

Authors: Rolla G. Bertero MT. Pastena G. Tartaglia N. Corradi F. Casabona R. Motta M. Caligaris-Cappio F.

Institution: Department of Biomedical Sciences and Human Oncology, Clinical Immunology and Haematology Division, Ospedale Mauriziano Umberto I di Torino, University of Torino, Largo Turati 62, 10128, Torino, Italy. grolla@mauriziano.it

Title: Primary lymphoma of the heart. A case report and review of the literature. [Review] [13 refs]

Source: Leukemia Research. 26(1):117-20, 2002 Jan.

Abstract: Primary cardiac lymphoma (PCL) is a rare and usually fatal neoplasm, which may cause syncope, arrhythmia, heart failure and pericardial effusion as presenting clinical complaints. A case of PCL in a 72-year-old man with moderate aortic stenosis is presented. The patient was investigated because of pericardial effusion and diagnosis of diffuse large B-cell lymphoma was obtained by open-chest biopsy of the heart. Fatal ventricular arrhythmia developed the day after the first course of chemotherapy. Clinical presentations and diagnostic approach of this rare tumour are discussed. While chemotherapy is the only effective treatment of PCL, early post-chemotherapy phase should be considered critical in patients with PCL, as suggested by other reported fatal complications in this period. [References: 13]

Publication Type: Case Reports. Journal Article. Review. Review, Tutorial.

<6>

Unique Identifier [PMID]: 7605969

Authors: Versluis PJ. Lamers RJ. van Belle AF.

Institution: Department of Diagnostic Radiology, University Hospital Maastricht, The Netherlands.

Title: Primary malignant lymphoma of the heart: CT and MRI features. [Review] [10 refs]

Source: Rofo: Fortschritte auf dem Gebiete der Rontgenstrahlen und der Nuklearmedizin. 162(6):533-4, 1995 Jun.

Publication Type: Case Reports. Journal Article. Review. Review of Reported Cases.

<7>

Unique Identifier [PMID]: 8107663

Authors: Pavlidis NA. Elisaf M. Bai M. Tolis C. Papadimitriou C.

Institution: Department of Medicine, Medical School, University of Ioannina, Greece.

Title: Primary lymphoma of the pericardium: report on a "cured" case and review of the literature. [Review] [33 refs]

Source: Medical & Pediatric Oncology. 22(4):287-91, 1994.

Abstract: This report describes a 62-year-old man with a primary diffuse, immunohistochemically proven B-cell lymphoma of large non-cleaved cell (centroblastic) type of the pericardium. The patient responded completely to systemic chemotherapy and remains free of disease 30 months after diagnosis. The use of non-cardiotoxic drugs in divided doses as initial treatment is emphasized. In addition, the authors reviewed the literature of the last decade regarding the management and outcome of patients with primary cardiac lymphomas. [References: 33]

Publication Type: Case Reports. Journal Article. Review. Review of Reported Cases.

<8>

Unique Identifier [PMID]: 11450997

Authors: Tai CJ. Wang WS. Chung MT. Liu JH. Chiang CY. Yen CC. Fan FS. Chiou TJ. Chen PM.

Institution: Department of Medicine, Veterans General Hospital-Taipei and National Yang-Ming University School of Medicine, Taiwan. cjtai@vghtpe.gov.tw

Title: Complete atrio-ventricular block as a major clinical presentation of the primary cardiac lymphoma: a case report. [Review] [19 refs]

Source: Japanese Journal of Clinical Oncology. 31(5):217-20, 2001 May.

Abstract: It is uncommon for malignant lymphomas to present primarily with cardiac invasion as the main clinical feature. What we are interested in is not only where the disease is, but also those symptoms that it may induce. Sudden onset of complete atrio-ventricular block is one of the most common clinical presentations. Cardiac tamponade is another common disease entity which, if it locates over outlets of great vessels, may also exert symptoms of obstructive vessels. Diagnosis can be made by needle aspiration under the guidance of transcutaneous or transesophageal echocardiography. Both CT scan and MRI play positive roles in the diagnosis of cardiac lymphomas, and the latter can even provide much more image information than the former. We report a 70-year-old male with primary cardiac lymphoma with initial clinical pictures of sudden onset of complete atrio-ventricular block. Chemotherapy was utilized with cyclophosphamide, vincristine and prednisolone (COP) initially for four courses and followed by adding doxorubicin (CHOP) for another three courses. The patient was still in remission status after treatment for 2 years. [References: 19]

Publication Type: Case Reports. Journal Article. Review. Review of Reported Cases.

<9>

Unique Identifier [PMID]: 11316130

Authors: Kamiya H. Yasuda T. Nagamine H. Sakakibara N. Nishida S. Kawasuji M. Watanabe G.

Institution: Department of Surgery, Kanazawa Univrsity School of Medicine, Japan. kamiya88@sf.m.kanazawa-u.ac.jp

Title: Surgical treatment of primary cardiac tumors: 28 years' experience in Kanazawa University Hospital. [Review] [26 refs]

Source: Japanese Circulation Journal. 65(4):315-9, 2001 Apr.

Abstract: To examine the clinical features of primary cardiac tumors, 34 patients who underwent surgical treatment from 1973 to 2000 at the Kanazawa University Hospital were analyzed and the literature was reviewed. The 34 patients were divided into 3 categories: (i) myxomas; (ii) benign non-myxomas; and (iii) malignant tumors. Twenty-three patients (70%) were diagnosed with myxomas, including 22 left atrial myxomas and 1 right atrial myxoma. Seven patients (18%) were diagnosed with benign non-myxoma tumors, including 3 hemangiomas, 1 fibroma, 1 rhabdomyoma, 1 pheochromocytoma, and 1 lipoma. Four patients (12%) were diagnosed with malignant tumors, including 2 angiosarcomas, 1 rhabdomyosarcoma, and 1 malignant fibrous histiocytoma. Among the myxoma patients, in-hospital mortality was 9% (2/23), late mortality was 10% (2/21), and no recurrent myxomas have been identified. Among benign non-myxoma patients there were no perioperative deaths; however, 1 patient died 11 years after surgery, with no linked cause. No recurrent tumors have been identified. Among malignant tumor patients, 1 patient died the day following surgery and the rest died within 14 months. Early and late results of surgery were acceptable for those patients with benign tumors, while the prognosis for patients with malignant tumors was very poor. [References: 26]

Publication Type: Journal Article. Review. Review, Multicase. Review, Tutorial.

<10>

Unique Identifier [PMID]: 10903697

Authors: Grebenc ML. Rosado de Christenson ML. Burke AP. Green CE. Galvin JR.

Institution: Department of Radiology, National Naval Medical Center, Bethesda, MD, USA.

Title: Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. [Review] [115 refs]

Source: Radiographics. 20(4):1073-103; quiz 1110-1, 1112, 2000 Jul-Aug.

Abstract: Primary cardiac and pericardial neoplasms are rare lesions and include both benign and malignant histologic types. Myxoma is the most frequent primary cardiac neoplasm, but other benign tumors include papillary fibroelastoma, rhabdomyoma, fibroma, hemangioma, lipoma, and paraganglioma. Cardiac sarcoma represents the second most common primary cardiac neoplasm. Lymphoma can also affect the heart primarily. Pericardial tumors that affect the heart include benign teratomas and malignant mesotheliomas. Patients affected with cardiac or pericardial neoplasms often present with cardiovascular compromise or embolic phenomena and exhibit cardiomegaly at chest radiography. Benign cardiac tumors typically manifest as intracavitary, mural, or epicardial focal masses, whereas malignant tumors demonstrate invasive features and may involve the heart diffusely. Benign lesions can usually be successfully excised, but patients with malignant lesions have an extremely poor prognosis. [References: 115]

Publication Type: Journal Article. Review. Review, Tutorial.

<11>

Unique Identifier [PMID]: 10807431

Authors: Vander Salm TJ.

Institution: Division of Cardiothoracic Surgery, University of Massachusetts Medical School, Worcester 01655-0304, USA.

Title: Unusual primary tumors of the heart. [Review] [69 refs]

Source: Seminars in Thoracic & Cardiovascular Surgery. 12(2):89-100, 2000 Apr.

Abstract: Primary tumors of the heart, with the exception of atrial myxomas, occur rarely; tumors metastatic to or directly invasive of the heart are far more common. About 75% of primary tumors are benign, and 75% of these are atrial myxomas. The benign tumors include rhabdomyomas, fibromas, papillary fibroelastomas, hemangiomas, pericardial cysts, lipomas, hamartomas, teratomas, mesotheliomas, and paragangliomas or pheochromocytomas. The last 3 may also be malignant. The malignant tumors consist of various sarcomas: myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, undifferentiated sarcoma, reticulum cell sarcoma, neurofibrosarcoma, and malignant fibrous histiocytoma. Cardiac tumors produce a large variety of symptoms through any of 4 mechanisms. Their mass can obstruct intracardiac blood flow or interfere with valve function. Local invasion can lead to arrhythmias or pericardial effusions with tamponade. Bits of tumor can embolize, causing systemic deficits when the tumors are on the left side of the heart. Finally, the tumors may cause systemic or constitutional symptoms. Some tumors, of course, produce no symptoms and become evident as incidental findings. The most useful diagnostic tool is the echocardiogram, which in almost all cases precisely locates the tumor and defines its extent. The echocardiographic appearance may also allow quite accurate prediction of the tumor type and whether it is malignant or benign. Magnetic resonance imaging serves as the next most important test where the density of T1 and T2 images may allow tumor cell type identification. With few exceptions, these tumors require operative excision. Most benign tumors can be resected completely; a few, because of their large size, cannot be, and only tumor debulking may be possible. Heart transplantation should be considered for these patients. Many of the malignant tumors cannot be resected completely, either because of the extent of local spread and invasion or because of the frequent distant metastases. Transplantation may also be an option for those with extensive local disease. The long-term results for resected benign tumors are excellent; the long-term results for sarcomas are very poor, and there are few survivors. For patients with unresectable sarcomas, radiation and chemotherapy may be used, but without great expectation of successful results. [References: 69]

Publication Type: Journal Article. Review. Review, Tutorial.

<12>

Unique Identifier [PMID]: 10555666

Authors: Araoz PA. Eklund HE. Welch TJ. Breen JF.

Institution: Department of Diagnostic Radiology, Mayo Clinic, Rochester, MN 55905, USA.

Title: CT and MR imaging of primary cardiac malignancies. [Review] [101 refs]

Source: Radiographics. 19(6):1421-34, 1999 Nov-Dec.

Abstract: Primary cardiac malignancies are rare tumors that are difficult to diagnose clinically. Different primary cardiac malignancies may have different clinical, morphologic, and radiologic features and intracardiac locations. Angiosarcoma is the most common primary cardiac malignancy. It tends to occur in the right atrium and involve the pericardium. Because of its tendency to hemorrhage, angiosarcoma often demonstrates areas of increased signal intensity with T1-weighted sequences. Undifferentiated sarcomas typically occur in the left atrium and have variable epidemiologic and radiologic features. Rhabdomyosarcoma is the most common primary cardiac malignancy in children and is more likely than other primary cardiac sarcomas to involve the valves. Primary cardiac osteogenic sarcoma almost always occurs in the left atrium and frequently demonstrates calcification. Certain features (eg, broad base of attachment, origin at a site other than the atrial septum) help differentiate this tumor from left atrial myxoma. Leiomyosarcoma favors the left atrium and tends to invade the pulmonary veins and mitral valve. Fibrosarcoma also tends to occur in the left atrium and is often necrotic. Liposarcoma is very rare and usually manifests as a large, infiltrating mass. Foci of macroscopic fat are occasionally seen. Primary cardiac lymphoma occurs more commonly in immunocompromised patients, frequently involves the pericardium, and, unlike other primary cardiac malignancies, may respond to chemotherapy. The advent of cross-sectional imaging has allowed earlier detection of primary cardiac malignancies as well as more accurate diagnosis and characterization. [References: 101]

Publication Type: Journal Article. Review. Review, Tutorial.

<13>

Unique Identifier [PMID]: 9338475

Authors: Ceresoli GL. Ferreri AJ. Bucci E. Ripa C. Ponzoni M. Villa E.

Institution: Department of Radiochemotherapy, Ospedale San Raffaele, Milan, Italy.

Title: Primary cardiac lymphoma in immunocompetent patients: diagnostic and therapeutic management. [Review] [77 refs]

Source: Cancer. 80(8):1497-506, 1997 Oct 15.

Abstract: BACKGROUND: Primary cardiac lymphoma (PCL) is extremely rare in immunocompetent patients. Different definition criteria have been employed in published series. Prognosis is poor due to diagnostic delay and relevance of the site of disease. METHODS: Two cases observed at the study institution are reported, with a review of 48 cases published in the literature from 1980 to 1996. Only patients with lymphoma confined to the heart and/or pericardium and those with a single and asymptomatic extracardiac site were considered for analysis. RESULTS: Eight patients had minimal extracardiac disease. The most common presentation was unresponsive heart failure. Electrocardiography findings were not specific. PCL usually arose in the right chambers as a mass, with or without pericardial effusion (> 80%). Chest X-rays, transthoracic echocardiography, and computed tomography scans are standard in diagnostic workup, but transesophageal echocardiography (TEE) and magnetic resonance imaging (MRI) showed a sensitivity > 90%. Cytology of pericardial effusion was diagnostic in 67% of cases. Thoracotomy was diagnostic in all cases, whereas less invasive procedures had high false-negative rates. Gross resection has no role. Early anthracycline-containing chemotherapy appears to improve survival, whereas the role of radiotherapy has not yet been defined. CONCLUSIONS: The diagnosis of PCL should be considered in patients with a cardiac mass and/or unexplained refractory pericardial effusion. Adequate diagnostic workup, including TEE and MRI, allows confirmation of the early suspicion of PCL. In the absence of a diagnostic cytology, an open biopsy may be indicated to avoid treatment delay. There is no evidence that PCL should be treated differently from other bulky aggressive lymphomas arising at other anatomic sites. [References: 77]

Publication Type: Case Reports. Journal Article. Review. Review of Reported Cases.

<14>

Unique Identifier [PMID]: 9295010

Authors: Roberts WC.

Title: Primary and secondary neoplasms of the heart. [Review] [87 refs]

Source: American Journal of Cardiology. 80(5):671-82, 1997 Sep 1.

Publication Type: Editorial. Review. Review, Tutorial.

<15>

Unique Identifier [PMID]: 9160076

Authors: Nakchbandi IA. Day HJ.

Institution: Department of Medicine, Abington Memorial Hospital, PA, USA.

Title: Primary cardiac lymphoma: initial symptoms suggestive of gastrointestinal disease. [Review] [32 refs]

Source: Southern Medical Journal. 90(5):539-43, 1997 May.

Abstract: We report a case of primary cardiac lymphoma in a patient who was not infected with human immunodeficiency virus and had symptoms suggestive of upper gastrointestinal (GI) disease. Examination revealed no GI abnormalities. Echocardiography, prompted by sudden development of congestive heart failure, revealed a large right atrial mass. Cardiac biopsy followed by staging evaluation indicated primary cardiac non-Hodgkin's lymphoma. After chemotherapy was begun, the tumor shrunk and GI symptoms resolved, suggesting an etiologic relationship by a referred pain mechanism. Unusual features of our case include the initial predominance of GI symptoms and the circumstances that led to diagnosis antemortem. The case also underscores the importance of considering intrathoracic disease in patients with upper abdominal symptomatology. [References: 32]

Publication Type: Case Reports. Journal Article. Review. Review of Reported Cases.

<16>

Unique Identifier [PMID]: 8947350

Authors: Cina SJ. Smialek JE. Burke AP. Virmani R. Hutchins GM.

Institution: Johns Hopkins Hospital, Baltimore, MD, USA.

Title: Primary cardiac tumors causing sudden death: a review of the literature. [Review] [59 refs]

Source: American Journal of Forensic Medicine & Pathology. 17(4):271-81, 1996 Dec.

Abstract: Sudden unexpected death accounts for 200,000-400,000 deaths each year in the United States. Although the vast majority of these fatalities are related to atherosclerotic heart disease, a small percentage (approximately 0.0025%) stem from primary cardiac neoplasms. There have been 120 cases of sudden death attributed to primary cardiac tumors in the (published) literature. Although 103 of these lesions were histologically benign (86%), their intracardiac locations precipitated conductive and hemodynamic abnormalities that resulted in sudden death. These tumors are usually easily recognized at necropsy. The most common intracardiac lesion causing sudden death, endodermal heterotopia of the atrioventricular (AV) node, however, may not be discovered unless the AV node is microscopically examined. Owing to the rarity of these neoplasms, a brief review of their salient gross and microscopic features is in order. [References: 59]

Publication Type: Journal Article. Review.

<17>

Unique Identifier [PMID]: 7924524

Authors: Aboulafia DM. Bush R. Picozzi VJ.

Institution: Section of Hematology and Oncology, University of Washington, Seattle.

Title: Cardiac tamponade due to primary pericardial lymphoma in a patient with AIDS. [Review] [34 refs]

Source: Chest. 106(4):1295-9, 1994 Oct.

Abstract: Cardiac tamponade due to lymphomatous involvement of the heart is a dramatic and unusual complication. Because of their nonspecific clinical presentation, these tumors are seldom diagnosed antemortem. We report the case of a patient with AIDS who presented with signs and symptoms of cardiac tamponade. Emergency pericardiocentesis followed by staging studies revealed large cell B-lymphocyte lymphoma confined to the pericardial space. With combination chemotherapy, a durable complete response was obtained. This case illustrates the potential benefit of aggressive treatment of extranodal non-Hodgkin's lymphoma in a patient with AIDS. The case is of particular interest because of the unusual development of isolated pericardial involvement as the sentinel sign of lymphoma. [References: 34]

Publication Type: Case Reports. Journal Article. Review. Review of Reported Cases.

<18>

Unique Identifier [PMID]: 2237901

Authors: Blondeau P.

Institution: Broussais Hospital, Paris, France.

Title: Primary cardiac tumors--French studies of 533 cases. [Review] [0 refs]

Source: Thoracic & Cardiovascular Surgeon. 38 Suppl 2:192-5, 1990 Aug.

Abstract: The present study combining the Eisenman French Joint Study and the Broussais Hospital experience is based upon 533 primary cardiac tumors which were operated on in France and by French-speaking teams during a period of about 25 years. The diagnosis of heart tumors was facilitated by the progressive development of new examination techniques. Echocardiography seems to be the simplest, safest, and the most reliable diagnostic method and is used more frequently then angiocardiography. Cardiac tumors occur at every age with a prevalence for females. About 10% of the surgically treated primary cardiac tumors were malignant (53 patients) with a constantly poor prognosis. The mean survival time of this collective after operation was only 1.5 years. 8% of the benign cardiac tumors were non-myxomatous (36), including fibromas, rhabdomyomas, and hamartomas. The mean age of this group was lower (21 years) than in the collectives with myxomas or with malignant cardiac tumors. Another characteristic of non-myxomatous tumors is the involvement of the ventricles by the neoplasm (88%). Complete resection of the tumor was possible in 75%, only 3 operative deaths occurred. Myxomas are the most common tumors of the heart, representing 83% of all primary cardiac tumors (444). Female patients were involved more often than males. The age of the patients ranged widely from 2.5 to 82 years. The typical localization of the myxomas was the left atrium (83%), followed by the right atrium (11%). In 5 cases myxomas were found multilocalized. Very often myxomas were mimicing valvular disease. In more than 30% peripheral embolization provoked the diagnosis. Only in 2% was the tumor totally asymptomatic.(ABSTRACT TRUNCATED AT 250 WORDS) [References: 0]

Publication Type: Journal Article. Review. Review, Multicase.

<19>

Unique Identifier [PMID]: 2658447

Authors: Castelli MJ. Mihalov ML. Posniak HV. Gattuso P.

Institution: Department of Pathology, Loyola University Medical Center, Maywood, IL 60153.

Title: Primary cardiac lymphoma initially diagnosed by routine cytology. Case report and literature review. [Review] [14 refs]

Source: Acta Cytologica. 33(3):355-8, 1989 May-Jun.

Abstract: A case of primary cardiac lymphoma initially diagnosed by routine cytologic examination of pericardial fluid is presented. In a 64-year-old woman woman who originally presented with chest pain and heart block, the initial clinical impression was ischemic heart disease. However, coronary angiography failed to reveal significant disease. An echocardiogram demonstrated pericardial fluid, which was drained. A small amount was sent for cytologic examination, and the diagnosis of malignant lymphoma, large cell type, was made. Subsequent radiologic examinations revealed an intracardiac mass involving the atrioventricular canal; surgical biopsy confirmed the diagnosis of a large cell lymphoma. While primary malignant lymphoma of the heart is rare, this case highlights the efficacy of routine cytologic examination of an effusion fluid (often drained therapeutically) in establishing the correct diagnosis. [References: 14]

Publication Type: Case Reports. Journal Article. Review. Review of Reported Cases.

<20>

Unique Identifier [PMID]: 6305485

Authors: Chou ST. Arkles LB. Gill GD. Pinkus N. Parkin A. Hicks JD.

Title: Primary lymphoma of the heart. A case report. [Review] [19 refs]

Source: Cancer. 52(4):744-7, 1983 Aug 15.

Abstract: The necropsy findings of a large cell lymphoma involving only the pericardium and myocardium in a 62-year-old woman are reported. The initial presenting symptoms were heart failure followed by rapidly progressive heart block. The diagnosis of cardiac lymphoma was suggested by gallium and blood pool isotope studies, and was subsequently confirmed by operative myocardial biopsy. The clinical course was abrupt, and the patient died before therapy was instituted. While primary cardiac lymphoma is an extremely rare condition, experience in this case suggests that noninvasive isotope studies, particularly gallium and blood pool, are helpful in the diagnosis of atypical cardiomyopathy. [References: 19]

Publication Type: Case Reports. Journal Article. Review.

<21>

Unique Identifier [PMID]: 230012

Authors: McAllister HA Jr.

Title: Primary tumors and cysts of the heart and pericardium. [Review] [33 refs]

Source: Current Problems in Cardiology. 4(2):1-51, 1979 May.

Publication Type: Journal Article. Review.

<22>

Unique Identifier [PMID]: 4589961

Authors: Fine G.

Title: Primary tumors of the pericardium and heart. [Review] [92 refs]

Source: Cardiovascular Clinics. 5(1):207-38, 1973.

Publication Type: Journal Article. Review.

<23>

Unique Identifier [PMID]: 4550797

Authors: McDevitt HO. Bodmer WF.

Title: Protein clinical manifestations of primary tumors of the heart. [Review] [41 refs]

Source: American Journal of Medicine. 52(1):1-8, 1972 Jan.

Publication Type: Journal Article. Review.

<24>

Unique Identifier [PMID]: 15671623

Authors: Fujisaki J. Tanaka T. Kato J. Saito T. Yano K. Shimizu Y. Sada T. Kitazume K. Fujita A. Kira Y.

Institution: Department of Cardiology, Showa General Hospital, Tokyo, Japan.

Title: Primary cardiac lymphoma presenting clinically as restrictive cardiomyopathy.

Source: Circulation Journal. 69(2):249-52, 2005 Feb.

Abstract: 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.

Publication Type: Case Reports. Journal Article.

<25>

Unique Identifier [PMID]: 15505107

Authors: Binder J. Pfleger S. Schwarz S.

Institution: Department of Neurology, Universitatsklinikum Mannheim, Theodor Kutzer Ufer 1-3, D-68135 Mannheim, Germany. j.binder@neuro.ma.uni-heidelberg.de

Title: Images in cardiovascular medicine. Right atrial primary cardiac lymphoma presenting with stroke.

Source: Circulation. 110(17):e451-2, 2004 Oct 26.

Publication Type: Case Reports. Journal Article.

<26>

Unique Identifier [PMID]: 15512827

Authors: Zakynthinos E. Tassopoulos G. Haritos C. Kitsanta P. Pyros J. Roussos C. Papiris SA.

Institution: Department of Critical Care and Pulmonary Services, National and Kapodistrian University of Athens, Athens, Greece. ezakynth@yahoo.com

Title: Huge biatrial primary cardiac B-cell lymphoma resulting in bilateral atrioventricular valve obstruction.

Source: Leukemia & Lymphoma. 45(11):2339-42, 2004 Nov.

Abstract: We report the case of a 48-year-old man with a clinical syndrome caused by both pulmonary and systemic venous congestion. Echocardiography indicated a huge tumor occupying both dilated atria almost completely, seeming to invade from the atrial septum. A test for HIV was negative. Our primary diagnosis was a biatrial myxoma. Urgent surgery was performed disclosing large cell B-cell primary cardiac lymphoma. The patient died 8 days later. Primary cardiac lymphoma is extremely rare in immunocompetent patients. Its rarity and heterogenous clinical presentation make diagnosis difficult. As tissue characterization is still beyond the capabilities of currently available echocardiography, a combination of examinations are indicated, when the clinical condition is relatively stable, in order to reach the exact diagnosis.

Publication Type: Case Reports. Journal Article.

<27>

Unique Identifier [PMID]: 14989742

Authors: Ikeda H. Nakamura S. Nishimaki H. Masuda K. Takeo T. Kasai K. Ohashi T. Sakamoto N. Wakida Y. Itoh G.

Institution: First Department of Pathology, Aichi Medical University, Aichi, Japan. hikeda@aicha-med-u.ac.jp

Title: Primary lymphoma of the heart: case report and literature review.

Source: Pathology International. 54(3):187-95, 2004 Mar.

Abstract: Primary cardiac lymphoma (PCL) is a rare and usually fatal neoplasma. A case of PCL in a 78-year-old man who complained of exertional dyspnea and peripheral edema is presented. Echocardiography revealed a mass in the right atrium and a diagnosis of low-grade B-cell lymphoma was obtained with the surgically resected tumor. The lesion appeared to have originated in the right atrium and involved the right ventricle. The patient died of bronchopneumonia 8 months after the initial consultation. The present case and 39 patients with PCL reported between 1995 and 2002 were reviewed. Forty patients showed various and non-specific symptoms such as dyspnea, edema, arrhythmia and pericardial effusion. Primary cardiac lymphoma occurred slightly more often in male patients (M : F = 23:17) and in the elderly in general (mean age, 67 years), with lesions found in the following locations, listed in order of frequency: right atrium, pericardium, right ventricle, left atrium, left ventricle, and other sites. Antemortem diagnosis was obtained in 37 of the 40 patients. Thirty-seven cases were of B-cell lineage and two cases were of T-cell lineage. Complete remission was obtained in only 15 of the 40 patients. Although PCL antemortem diagnoses have been made in the majority of recent cases, the prognosis still remains poor.

Publication Type: Case Reports. Journal Article.

<28>

Unique Identifier [PMID]: 15226345

Authors: Maier BO. von Scheidt W. Sciuk J. Sumer C. Leipprand E. Schlimok G. Sandherr M.

Institution: Department of Hematology and Oncology, Institute of Pathology, Klinikum Augsburg, Augsburg, Germany.

Title: Cardiac involvement in malignancies. Case 4. Primary cardiac diffuse large B-cell lymphoma: diagnosis by transesophageal echocardiography-guided transvenous biopsy.

Source: Journal of Clinical Oncology. 22(13):2745-7, 2004 Jul 1.

Publication Type: Case Reports. Journal Article.

<29>

Unique Identifier [PMID]: 14745155

Authors: Nakagawa Y. Ikeda U. Hirose M. Ubukata S. Katsuki TA. Kaminishi Y. Saito T. Hironaka M. Izumi T. Shimada K.

Institution: Division of Cardiovascular Medicine, Jichi Medical School, Tochigi, Japan.

Title: Successful treatment of primary cardiac lymphoma with monoclonal CD20 antibody (rituximab).

Source: Circulation Journal. 68(2):172-3, 2004 Feb.

Abstract: Primary cardiac malignant lymphoma is extremely rare and almost all patients die within weeks. Monoclonal CD20 antibody (rituximab) was administered to a patient with primary cardiac B-cell non-Hodgkin's lymphoma expressing a CD20 molecule. The results suggest that rituximab may be a safe and effective new therapy for primary cardiac B-cell lymphoma.

Publication Type: Case Reports. Journal Article.

<30>

Unique Identifier [PMID]: 14768493

Authors: Timoteo AT. Gouveia R. Goncalves PA. Trabulo M. Ribeiras R. Canada M. Gil V. Martins AP. Silva JA. Seabra-Gomes R.

Institution: Servicos de Cardiologia e Anatomia Patologica, Hospital de Santa Cruz, Carnaxide, Portugal. ana_timoteo@yahoo.com

Title: Lymphoma with clinical presentation of a primary pericardial tumor.

Source: Revista Portuguesa de Cardiologia. 22(11):1385-91, 2003 Nov.

Abstract: Primary malignant cardiac tumors, particularly lymphoma, are rare entities. Cardiac involvement or metastization of the heart from neoplasia located elsewhere are more frequently found. We present the case of a 79-year-old patient admitted with heart failure symptoms with a 3-week evolution. Evaluation led to the identification of a cardiac tumor with unusual clinical presentation and with a rapid and fatal evolution. Pathologic analysis identified a B-cell non-Hodgkin lymphoma.

Publication Type: Case Reports. Journal Article.

<31>

Unique Identifier [PMID]: 14611830

Authors: Timperley J. Mitchell AR. Becher H.

Institution: Department of Cardiology, The John Radcliffe Hospital, Headington, Oxford, UK. cardiology.registrars@ohr.nhs.uk

Title: Primary cardiac lymphoma.

Source: European Journal of Echocardiography. 4(4):327-30, 2003 Dec.

Abstract: We present a case of primary cardiac lymphoma, which is a rare condition compared with secondary metastatic involvement and is associated with poor prognosis. This case demonstrates the use of transthoracic echocardiography for the assessment of tumour regression in response to chemotherapy.

Publication Type: Case Reports. Journal Article.

<32>

Unique Identifier [PMID]: 14515922

Authors: Hayes D Jr. Liles DK. Sorrell VL.

Institution: Section of General Internal Medicine, Department of Internal Medicine, Brody School of Medicine, East Carolina University, Greenville, NC, USA.

Title: An unusual cause of new-onset atrial flutter: primary cardiac lymphoma.

Source: Southern Medical Journal. 96(8):799-802, 2003 Aug.

Abstract: Primary cardiac lymphoma is a rare disease with a high mortality rate due to the advanced stage of myocardial involvement at presentation. The diagnosis is extremely difficult to make because of the rarity of the disease, variability of clinical manifestations, limited noninvasive diagnostic techniques available, and difficulties and/or delays in the use of invasive measures. The incidence of the disease is increasing, especially among immunocompromised patients, with those suffering from acquired immunodeficiency syndrome accounting for the greatest increase. We report the case of an immunocompetent 76-year-old black woman who presented with near-syncopal episodes. Transthoracic echocardiogram revealed a right atrial mass. Surgical resection was performed, and a diagnosis of large B-cell non-Hodgkin's lymphoma was made. Primary cardiac lymphoma should be considered in any patient with a cardiac mass. Prompt diagnosis and treatment of primary cardiac lymphoma is imperative for survival.

Publication Type: Case Reports. Journal Article.

<33>

Unique Identifier [PMID]: 12560231

Authors: Quigley MM. Schwartzman E. Boswell PD. Christensen RL. Gleason LA. Sharpe RW. D'Amato TA.

Institution: Department of Pathology, Division of Cardiology, Naval Medical Center, San Diego, CA 92134, USA. mmquigley@nmcsd.med.navy.mil

Title: A unique atrial primary cardiac lymphoma mimicking myxoma presenting with embolic stroke: a case report.

Source: Blood. 101(12):4708-10, 2003 Jun 15.

Abstract: An immunocompetent 29-year-old male presented with an embolic stroke from an unusual primary cardiac lymphoma. The cardiac lesion consisted of a polypoid, left atrial, mural fibrin thrombus with anaplastic tumor cells lining the surface of the clot. Histologic, immunophenotypic, and molecular characterizations were consistent with a diagnosis of CD30+ large B-cell lymphoma with anaplastic cytology. While tumor emboli from invasive primary cardiac lymphomas have been reported, this noninvasive fibrin thrombus-associated lymphoma appears to be unique and previously unreported.

Publication Type: Case Reports. Journal Article.

<34>

Unique Identifier [PMID]: 11952118

Authors: Saotome M. Yoshitomi Y. Kojima S. Kuramochi M.

Institution: Division of Cardiology, Tohsei National Hospital, Shizuoka, Japan.

Title: Primary cardiac lymphoma--a case report.

Source: Angiology. 53(2):239-41, 2002 Mar-Apr.

Abstract: 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.

Publication Type: Case Reports. Journal Article.

<35>

Unique Identifier [PMID]: 11519090

Authors: Ryu SJ. Choi BW. Choe KO.

Institution: Department of Diagnostic Radiology, and Institute of Radiological Science, Yonsei University, College of Medicine, C.P.O. Box 8044, Seoul 120-752, Korea.

Title: CT and MR findings of primary cardiac lymphoma: report upon 2 cases and review.

Source: Yonsei Medical Journal. 42(4):451-6, 2001 Aug.

Abstract: Although primary cardiac lymphoma was a near fatal disease in the past, recent advances in imaging diagnosis and chemotherapy have dramatically improved survival. We describe the CT and MR findings of primary cardiac lymphoma in two immunocompetent subjects who recovered after chemotherapy. The primary cardiac lymphomas manifested as a poorly enhancing mass on CT, relatively hyperintense on a T2-weighted image and a heterogeneously enhanced mass on contrast-enhanced MR. The tumor also showed a predilection for right heart chambers and contiguous infiltration along adjacent structures.

Publication Type: Case Reports. Journal Article.

<36>

Unique Identifier [PMID]: 11347623

Authors: Alter P. Grimm W. Tontsch D. Maisch B.

Title: Diagnosis of primary cardiac lymphoma by endomyocardial biopsy.

Source: American Journal of Medicine. 110(7):593-4, 2001 May.

Publication Type: Case Reports. Letter.

<37>

Unique Identifier [PMID]: 11214017

Authors: Mejhert M. Muller-Suur R.

Institution: Division of Internal Medicine, Karolinska Institutet Danderyd Hospital, Sweden. marit.mejhert@med.ds.sll.se

Title: Primary lymphoma of the heart.

Source: Scandinavian Cardiovascular Journal. 34(6):606-8, 2000 Dec.

Abstract: Primary tumors of the heart are rare. Most patients have very few clinical manifestations and present with uncharacteristic symptoms, often misdiagnosed as heart failure. As investigation techniques such as echocardiography, computed tomography and magnetic resonance imaging become more widespread, the clinician will be confronted with the diagnosing of cardiac masses. Early identification is essential for optimum treatment. We present a case of malignant lymphoma of the heart, where treatment led to remission from cardiac growth of the tumor.

Publication Type: Case Reports. Journal Article.

<38>

Unique Identifier [PMID]: 10790358

Authors: Burling F. Devlin G. Heald S.

Institution: Department of Cardiology, Waikato Hospital, Hamilton, New Zealand. devling@hwl.co.nz

Title: Primary cardiac lymphoma diagnosed with transesophageal echocardiography-guided endomyocardial biopsy.

Source: Circulation. 101(17):E179-81, 2000 May 2.

Publication Type: Case Reports. Journal Article.

<39>

Unique Identifier [PMID]: 10784641

Authors: Beckwith C. Butera J. Sadaniantz A. King TC. Fingleton J. Rosmarin AG.

Institution: The Miriam Hospital, Brown University School of Medicine, Providence, RI, USA.

Title: Diagnosis in oncology. Case 1: primary transmural cardiac lymphoma.

Source: Journal of Clinical Oncology. 18(9):1996-7, 2000 May.

Publication Type: Case Reports. Journal Article.

<40>

Unique Identifier [PMID]: 10716528

Authors: Miyashita T. Miyazawa I. Kawaguchi T. Kasai T. Yamaura T. Ito T. Takei M. Kiyosawa K.

Institution: Department of Internal Medicine, Kofu Municipal Hospital, Yamanashi, Japan.

Title: A case of primary cardiac B cell lymphoma associated with ventricular tachycardia, successfully treated with systemic chemotherapy and radiotherapy: a long-term survival case.

Source: Japanese Circulation Journal. 64(2):135-8, 2000 Feb.

Abstract: We experienced a long-term survival case of primary cardiac lymphoma (PCL) demonstrating ventricular tachycardia (VT) as an initial sign, which was related to localized myocardial damage by lymphoma cells. A 70-year-old woman with sustained VT was admitted to the Kofu Municipal Hospital. VT ceased with the administration of disopyramide intravenously. The origin of the VT was the free wall of the right ventricular outflow tract (RVOT) as observed by electrocardiography on admission. A solitary mass in the free wall of the RVOT was found by echocardiography, chest computed tomographic scanning and magnetic resonance imaging. There was no evidence of extracardiac involvement. The patient was histologically diagnosed as PCL by endomyocardial biopsy. Chemotherapy started immediately after the diagnosis and the mass showed a marked reduction in size. After 8 cycles of chemotherapy, radiotherapy was performed. Pericardial thickness in the free wall of the RVOT developed without severe side effects. Complete remission has been maintained for 30 months after the initial diagnosis, and no recurrence and arrhythmias have been detected during the follow-up period. It was demonstrated that rapid diagnosis and chemotherapy followed by radiotherapy for PCL achieved better survival.

Publication Type: Case Reports. Journal Article.

<41>

Unique Identifier [PMID]: 10559123

Authors: Skalidis EI. Parthenakis FI. Zacharis EA. Datseris GE. Vardas PE.

Institution: Department of Cardiology, University Hospital of Heraklion, Crete, Greece.

Title: Pulmonary tumor embolism from primary cardiac B-cell lymphoma.

Source: Chest. 116(5):1489-90, 1999 Nov.

Abstract: We report the case of a 54-year-old man with pulmonary embolism during convalescence from "idiopathic" pericarditis. A transthoracic echocardiographic examination indicated a large mass within the right atrium. Subsequently, he died from refractory hypotension. On autopsy, two large whitish nodules were found in the right atrium; there was also nodular epicardial infiltration. Both lungs showed multiple, grossly visible tumor emboli with pulmonary infarction and no evidence of conventional thromboembolism. This is the first report of pulmonary tumor embolism due to large cell B-cell primary cardiac lymphoma. Refractory unexplained pericardial effusion, pulmonary embolism without risk factors for venous thrombosis, and/or the existence of a mass in the right heart should arouse clinical suspicion for this rare malignancy.

Publication Type: Case Reports. Journal Article.

<42>

Unique Identifier [PMID]: 9869208

Authors: Begueret H. Labouyrie E. Dubus P. Sempe S. Goussot JF. Besse P. De Mascarel A. Merlio JP.

Institution: Department of Pathology, Hopital du Haut-Leveque, Bordeaux, France.

Title: Primary cardiac lymphoma in an immunocompetent woman.

Source: Leukemia & Lymphoma. 31(3-4):423-8, 1998 Oct.

Abstract: We report a fatal primary cardiac non-Hodgkin's lymphoma in a 62 years old immunocompetent woman presenting with tamponade and complete atrioventricular block. CT-scan, echocardiography and autopsy examination showed a tumor largely infiltrating the heart without extracardiac involvement. A surgical biopsy revealed high grade B-cell non-Hodgkin's lymphoma with a misleading myelomonocytic CD68 (KPI) expression. Polymerase Chain Reaction analysis revealed a clonal rearrangement of the immunoglobulin heavy chain gene and confirmed the B-cell origin of the lymphoma. Our report also emphasizes the role of immunohistochemical and molecular techniques in the diagnosis.

Publication Type: Case Reports. Journal Article.

<43>

Unique Identifier [PMID]: 9797085

Authors: Daus H. Bay W. Harig S. Schneider G. Feiden W. Schieffer H.

Institution: Department of Internal Medicine I, University Hospital, Homburg/Saar, Germany.

Title: Primary lymphoma of the heart: report of a case with histological diagnosis of the transvenously biopsied intracardiac tumor.

Source: Annals of Hematology. 77(3):139-41, 1998 Sep.

Abstract: Primary cardiac lymphoma is an extremely rare disease and is associated with a high mortality. In most cases, lymphomatous involvement of the heart and/or pericardium occurs as a late manifestation of disseminated disease. Primary cardiac lymphoma is treatable when appropriately diagnosed. We report the case of an immunocompetent 69-year-old patient who presented with signs of dyspnea and a transmural mass infiltrating the apical section of both ventricles. Examination of the tissue obtained by transvenous biopsy revealed high-grade non-Hodgkin's lymphoma of B-cell lineage. The patient was treated successfully with CHOP chemotherapy. This case demonstrates that early diagnosis and intensive chemotherapy might contribute to a better prognosis for patients with malignant lymphoma of the heart.

Publication Type: Case Reports. Journal Article.

<44>

Unique Identifier [PMID]: 9740487

Authors: Makishima H. Isobe M. Imamura H.

Institution: The First Department of Internal Medicine, Shinshu University School of Medicine, Asahi, Matsumoto, Japan.

Title: A case of primary cardiac lymphoma: utility of serum soluble interleukin-2 receptor for noninvasive diagnosis.

Source: International Journal of Cardiology. 65(3):291-3, 1998 Aug.

Abstract: A 70-year-old woman was referred to us for the investigation of a tumor mass in the right sided of the heart. LDH and soluble interleukin-2 receptor (sIL2r) were elevated. She died one day after the initiation of radiation therapy because of intractable cardiogenic shock. Primary cardiac lymphoma was identified at autopsy. Measurement of serum sIL2r lead us to the antemortem diagnosis of the disease.

Publication Type: Case Reports. Journal Article.

<45>

Unique Identifier [PMID]: 9681213

Authors: Murphy PT. Sivakumaran M. Coleby P.

Institution: Department of Haematology, Leicester Royal Infirmary, UK.

Title: Primary cardiac lymphoma: death from cardiac asystole after attaining second complete remission.

Source: Clinical & Laboratory Haematology. 20(1):57-9, 1998 Feb.

Abstract: A patient with primary high grade cardiac lymphoma, diagnosed by pericardial fluid cytology, achieved complete remission with chemotherapy. After a relapse 11 months later, a second complete remission was attained with radiotherapy and chemotherapy but the patient suffered a fatal cardiac asystole during intensification therapy. A review of the recent literature suggests that prognosis for this rare extra nodal lymphoma has markedly improved as a result of early diagnosis and prompt institution of aggressive antilymphoma therapy.

Publication Type: Case Reports. Journal Article.

<46>

Unique Identifier [PMID]: 9487479

Authors: Unger P. Kentos A. Cogan E. Renard M. Crasset V. Stoupel E.

Institution: Department of Cardiology, Erasme Hospital, Universite Libre de Bruxelles, Belgium.

Title: Primary cardiac lymphoma: diagnosis by transvenous biopsy under transesophageal echocardiographic guidance.

Source: Journal of the American Society of Echocardiography. 11(1):89-91, 1998 Jan.

Abstract: A 64-year-old woman presenting with dizziness and atrioventricular conduction disturbances was found to have a right atrial mass by two-dimensional transthoracic echocardiography. Transesophageal echocardiography allowed further delineation of the tumor and safe performance of transvenous biopsy, thereby obviating the need for surgery. Pathological examination of the biopsy specimen as well as the absence of extracardiac location established the diagnosis of primary cardiac lymphoma.

Publication Type: Case Reports. Journal Article.

<47>

Unique Identifier [PMID]: 9445177

Authors: Tada H. Asazuma K. Ohya E. Hayashi T. Nakai T. Nakayama T. Ueda T.

Institution: Third Department of Internal Medicine, Fukui Medical School, Izumigaoka Onsen Hospital, Japan.

Title: Images in cardiovascular medicine. Primary cardiac B-cell lymphoma.

Source: Circulation. 97(2):220-1, 1998 Jan 20.

Publication Type: Case Reports. Journal Article.

<48>

Unique Identifier [PMID]: 8651857

Authors: Ito M. Nakagawa A. Tsuzuki T. Yokoi T. Yamashita Y. Asai J.

Institution: Department of Pathology, Nagoya University Hospital, Japan.

Title: Primary cardiac lymphoma. No evidence for an etiologic association with Epstein-Barr virus.

Source: Archives of Pathology & Laboratory Medicine. 120(6):555-9, 1996 Jun.

Abstract: OBJECTIVES--To report two cases of primary cardiac lymphoma, a rare extranodal lymphoma with an unknown pathogenesis, and to compare them to secondary B-cell cardiac lymphoma. DESIGN--Clinicopathologic features are described, using histologic and immunophenotypic examinations. The Epstein-Barr virus genome is detected by in situ hybridization. PATIENTS--Of 80 autopsied cases of malignant lymphoma identified at Nagoya (Japan) University Hospital, two patients with primary cardiac lymphoma and five patients with secondary cardiac B-cell lymphoma were selected. RESULTS--None of the seven selected cases showed immunodeficiency, autoimmune disorders, or chronic inflammatory processes. Primary cardiac lymphomas had B-cell phenotypes with mu and lambda chain monoclonality. Immunostaining for Epstein-Barr virus (latent membrane protein-1) and Epstein-Barr virus-encoded RNA-1 in situ hybridization did not demonstrate an association of these lymphoma with Epstein-Barr virus infection. The majority of secondary cardiac B-cell lymphomas were extranodal lymphomas and extranodal or serosal involvement was more prominent than nodal involvement. CONCLUSION--These findings suggest that primary cardiac lymphoma, unlike pyothorax-associated pleural lymphoma, appears to have no association with chronic inflammation or Epstein-Barr virus infection.

Publication Type: Case Reports. Journal Article.

<49>

Unique Identifier [PMID]: 8619670

Authors: Sommers KE. Edmundowicz D. Katz WE. Hattler BG.

Institution: Division of Cardiothoracic Surgery, University of Pittsburgh Medical Center, Pittsburgh 15213, USA.

Title: Primary cardiac lymphoma: echocardiographic characterization and successful resection.

Source: Annals of Thoracic Surgery. 61(3):1001-3, 1996 Mar.

Abstract: We present a case of primary cardiac lymphoma in a patient with dyspnea and hypoxemia. Transesophageal echocardiography reveals a large right atrial mass and an atrial septal aneurysm with right-to-left shunting through a patent foramen ovale. The patient underwent resection and atrial reconstruction. Pathology was a B cell lymphoma with diffuse large cell histology. There was no evidence of extracardiac involvement, and the patient is well 3 months postoperatively with a normal transthoracic echocardiogram.

Publication Type: Case Reports. Journal Article.

<50>

Unique Identifier [PMID]: 8619669

Authors: Margolin DA. Fabian V. Mintz U. Botham MJ.

Institution: Department of Surgery, Case Western Reserve University School of Medicine, Cleveland 44106, USA.

Title: Primary cardiac lymphoma.

Source: Annals of Thoracic Surgery. 61(3):1000-1, 1996 Mar.

Abstract: A case of primary cardiac non-Hodgkin's B cell lymphoma is described in a patient presenting with obstructive right heart failure. Unlike the majority of cases, in this case the tumor was diagnosed ante mortem. THe patient's history combined with the aggressive use of noninvasive echocardiography are helpful in diagnosing this rare lesion.

Publication Type: Case Reports. Journal Article.

<51>

Unique Identifier [PMID]: 7571977

Authors: Chao TY. Han SC. Nieh S. Lan GY. Lee SH.

Institution: Department of Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, Republic of China.

Title: Diagnosis of primary cardiac lymphoma. Report of a case with cytologic examination of pericardial fluid and imprints of transvenously biopsied intracardiac tissue.

Source: Acta Cytologica. 39(5):955-9, 1995 Sep-Oct.

Abstract: BACKGROUND: Primary cardiac lymphoma (PCL) is a treatable disease when appropriately diagnosed. Therefore, a prompt, safe method with high diagnostic accuracy is prerequisite to successful therapy for PCL. CASE: A 57-year-old male presented with exertional dyspnea and atrial fibrillations. A pericardial effusion (PE) and several tumor masses occupying both atria were found. Cytologic examinations of PE and of imprints of the tissues obtained by transvenous biopsy of the cardiac tumors revealed numerous small, round tumor cells and lymphoglandular bodies, suggestive of malignant lymphoma. This cytologic impression was confirmed by immunocytochemical studies on the same cytologic material. Histologic studies reaffirmed the diagnosis of B-cell lymphoma. The patient received eight courses of chemotherapy, with complete remission of the illness. CONCLUSION: Cardiac lymphoma can be quickly and safely diagnosed by cytologic examination of PE or transvenously biopsied cardiac tissue, with confirmation by immunocytochemical studies. Exploratory thoracotomy for biopsy can be avoided.

Publication Type: Case Reports. Journal Article.

<52>

Unique Identifier [PMID]: 8107664

Authors: Stein M. Zyssman I. Kantor A. Spencer D. Lewis D. Bezwoda W.

Institution: Department of Medical Oncology and Hematology, Johannesburg Hospital, University of the Witwatersrand Medical School, Republic of South Africa.

Title: Malignant lymphoma with primary cardiac manifestations: a case report.

Source: Medical & Pediatric Oncology. 22(4):292-5, 1994.

Abstract: This case report describes a 29-year-old black patient presenting with right heart failure secondary to massive lymphomatous cardiac involvement. Additional sites of involvement included mesenteric lymph nodes and the small bowel. Malignant lymphoma with primary cardiac manifestations is very rare and follows an aggressive course. The importance of early diagnosis and commencement of immediate therapy is emphasized.

Publication Type: Case Reports. Journal Article.

<53>

Unique Identifier [PMID]: 8227590

Authors: Dorsay TA. Ho VB. Rovira MJ. Armstrong MA. Brissette MD.

Institution: Department of Radiology, Madigan Army Medical Center, Tacoma, WA 98431.

Title: Primary cardiac lymphoma: CT and MR findings.

Source: Journal of Computer Assisted Tomography. 17(6):978-81, 1993 Nov-Dec.

Abstract: Primary cardiac lymphomas, defined as those involving only the heart and pericardium, are extremely rare tumors. These tumors are commonly fatal and until recently were rarely diagnosed antemortem. We describe the CT and MR findings in a case of primary lymphoma of the heart currently responding to chemotherapy.

Publication Type: Case Reports. Journal Article.

<54>

Unique Identifier [PMID]: 1476062

Authors: Kasai K. Kuwao S. Sato Y. Murayama M. Harano Y. Kameya T.

Institution: Department of Pathology, Kitasato University School of Medicine, Kanagawa, Japan.

Title: Case report of primary cardiac lymphoma. The applications of PCR to the diagnosis of primary cardiac lymphoma.

Source: Acta Pathologica Japonica. 42(9):667-71, 1992 Sep.

Abstract: A 75-year-old man initially complained of pollakiuria and low abdominal pain, and died of massive bleeding from an exacerbated gastric ulcer. The diagnosis of primary cardiac lymphoma was made postmortem. The tumor involved only the epicardium and myocardium, which met the criteria of primary cardiac lymphoma as defined by the Armed Forces Institute of Pathology. The lymphoma consisted of large cells and expressed the B cell marker, CD20. Although chronic inflammation due to chronic renal failure was observed in the pericardium around the lymphoma, polymerase chain reaction (PCR) was conducted to detect monoclonality at the DNA level in lymphoma cells, which were shown to comprise a monoclonal population.

Publication Type: Case Reports. Journal Article.

<55>

Unique Identifier [PMID]: 2787096

Authors: Proctor MS. Tracy GP. Von Koch L.

Title: Primary cardiac B-cell lymphoma.

Source: American Heart Journal. 118(1):179-81, 1989 Jul.

Publication Type: Case Reports. Journal Article.

<56>

Unique Identifier [PMID]: 3493750

Authors: Guarner J. Brynes RK. Chan WC. Birdsong G. Hertzler G.

Title: Primary non-Hodgkin's lymphoma of the heart in two patients with the acquired immunodeficiency syndrome.

Source: Archives of Pathology & Laboratory Medicine. 111(3):254-6, 1987 Mar.

Abstract: We report two cases of primary cardiac lymphoma that developed in patients suffering from the acquired immunodeficiency syndrome. Both cases of lymphoma were histologically aggressive as generally observed in patients with the acquired immunodeficiency syndrome. The lymphoma cells in the center of a tumor nodule obtained from one patient were monoclonal B-cells, whereas those at the periphery showed a polyclonal pattern of staining. It is postulated that this represents a monoclonal lymphoma evolving from a polyclonal B-cell lymphoproliferation analogous to those reported in some cases of lymphoma in immunosuppressed patients infected with Epstein-Barr virus. The lymphoma cells in the other case failed to stain for cytoplasmic immunoglobulins. The possible underlying basis for the increase in incidence of lymphoma in immunodeficiency and the reasons for prevalence of extranodal sites are discussed.

Publication Type: Case Reports. Journal Article.

<57>

Unique Identifier [PMID]: 3485370

Authors: Gelman KM. Ben-Ezra JM. Steinschneider M. Dutcher JP. Keefe DL. Factor SM.

Title: Lymphoma with primary cardiac manifestations.

Source: American Heart Journal. 111(4):808-11, 1986 Apr.

Publication Type: Case Reports. Journal Article.