Primary lymphoma of the heart. Prolonged survival with early systemic therapy in a patient

Abstract

Primary lymphoma of the heart is an uncommon malignancy usually recognized at autopsy or fatal within a few weeks of diagnosis. Recently, it was reported in patients with acquired immune deficiency syndrome. A patient with diffuse large cell lymphoma of the heart is reported who had chest pain and rapidly evolving cardiac arrhythmias. The human immune deficiency virus antibody test was negative. Because of an aggressive diagnostic approach, therapy with cyclophosphamide, doxorubicin, vincristine, and prednisone was started on the third day after diagnosis. The patient has survived 18 months with an objective response. To the authors' knowledge, this is the longest reported survival in primary cardiac lymphoma. The diagnosis in this patient was aided by excellent tumor delineation by nuclear magnetic resonance scanning. The authors believe that better survival in this patient was a result of prompt diagnosis and treatment because the behavior of the lymphoma was similar to aggressive lymphomas arising elsewhere. Cancer 68:2289–2292, 1991.

Primary cardiac lymphoma in immunocompetent patients: a report of three cases and review of the literature

“…Furthermore, cytogenetic analysis could be performed on centesis fluid to analyze specific translocations. In our study, as well as in 40% of the reviewed cases, diagnosis of primary cardiac lymphoma was obtained by biopsy of cardiac tissue during an explorative thoracotomy or mediastinoscopy [1,3,6,7,9,15,19,23,28,29,32,35,38,39]. Less invasive procedures may be used in critically ill patients, small children, or preg- nant women and may provide diagnosis in 23% of cases.…”

“…Primary cardiac lymphoma remains asymptomatic until it produces a mass effect when the tumor obstructs cardiac output or causes local invasion, embolization, or systemic manifestations. Chest pain is the most common symptom occurring in 26% [6,10,18,27,28,29,30,31,38], dyspnea in 20% [7,8,9,28,34,36,37], superior vena caval syndrome in 9% [3,15,17] Chest X-ray is not usually helpful for the detection of cardiac tumor. It can reveal non-specific signs such as pleural effusion and/or cardiomegaly.…”

“…Drainage of pericardial effusion can have both therapeutic and diagnostic purposes [7]. A diagnostic cytologic sample is obtained in 14% of primary cardiac lymphoma [6,8,29,31,34]. It may be difficult to differentiate primary cardiac lymphoma from benign reactive lymphocytosis or other neoplasms by cytology alone.…”

“…It is an extranodal lymphoma involving only the heart and/or the pericardium [5]. We report 3 cases of primary cardiac lymphoma and review 35 cases from the literature [1,2,3,6,7,8,9,10,11,12,15,17,18,19,20,21,22,23,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,41].…”

Diffuse Large B-cell Lymphoma Diagnosed by Intracardiac Echocardiography-guided Cardiac Tumor Biopsy

“…Diagnostic delay and difficulty worsen the prognosis. However, recent reports suggest that an earlier diagnosis and appropriate therapy can improve the long-term prognosis (11). In general, a definitive diagnosis as PCL is confirmed by an endomyocardial, surgical excisional biopsy or pericardial fluid cytology.…”