An Unusual Cause of New-onset Atrial Flutter

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An Unusual Cause of New-onset Atrial Flutter
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.

Malignant neoplasms rarely occur in the heart, and primary tumors of pericardial and myocardial origin are even more uncommon. In autopsy series, the incidence of primary cardiac tumors varies between 0.0017% and 0.28%. Among 533 cases of primary tumors or cysts of the heart and pericardium reviewed by the Armed Forces Institute of Pathology, 60% were benign tumors, 17% were cysts, and 23% were malignant tumors. Tumors metastatic to the heart and pericardium are 20 to 30 times more common than primary lesions, because 10 to 20% of patients with known malignancy have secondary involvement at autopsy.

Primary cardiac lymphoma (PCL) is defined as non-Hodgkin's lymphoma (NHL) involving only the heart and/or the pericardium, as described by McAllister and Fenoglio. Few cases of PCL meeting this criterion have been reported. Cardiac involvement with disseminated NHL has been reported in approximately 20% of autopsy patients; however, PCL is a rare malignancy and is seldom recognized before death.

Early and accurate diagnosis is of the highest importance in initiating appropriate therapy to prolong survival. Diagnosis of cardiac tumors has been facilitated by the use of modern imaging, but histologic diagnosis is required, and access to the tumor is often difficult.

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