Extensive Primary Cardiac Lymphoma Diagnosed by Percutaneous Endomyocardial Biopsy

Abstract

Primary cardiac lymphoma (PCL) is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium. It is rare in immunocompetent patients and represents 1.3% of primary cardiac tumors and 0.5% of extranodal lymphomas. The clinical behavior is aggressive and the early symptoms are cardiac failure, syncope, arrhythmia, or pericardial effusion. Although echocardiography, computed tomography (CT) scan, magnetic resonance image (MRI) are the mainly used imaging techniques to detect cardiac tumors, pathologic examination is always required to confirm the diagnosis. Diagnosis of PCL is difficult due to non-specific clinical manifestations and requires invasive approach to get histopathologic evidence. While surgery with systemic chemotherapy or in combination with irradiation has been attempted, the only effective treatment is chemotherapy. However, the prognosis remains poor. We report on a 42-year-old woman who is diagnosed histopathologically as PCL by cardiac catheterization assisted percutaneous endomyocardial biopsy and treated successfully by anthracycline based chemotherapy.

Primary Cardiac Lymphoma in an Immunocompetent 71-Year-Old Man

“…To our knowledge, only 23 cases of PCL in immunocompetent patients have been reported to date (Table I). [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] According to these reports, no patient presented with noncardiac lymphomatous localization, but, as in our patient, it was impossible to absolutely exclude noncardiac involvement in the absence of autopsic investiga- Nine of these patients were treated with surgical excision followed by chemotherapy, and 5 of them survived at least 6 months after treatment. No evidence exists concerning the necessity of surgery as an adjunct to chemotherapy in PCL; moreover, in those 9 cases surgery was the final diagnostic step, along with excision as treatment.…”

Typical and Atypical Magnetic Resonance Imaging Appearances of Primary Cardiac Lymphoma

“…Chin et al [7] found that treatment with chemotherapy alone resulted in remission in almost 61% of patients, while surgery alone had no effect on outcome. Surgery buys time for chemotherapy to be of therapeutic value.…”

Broadening the scope of thoracic oncological intervention: a novel minimally invasive method for the diagnosis of primary cardiac lymphoma

“…It typically presents in elderly males and predominantly involves the right-sided cardiac chambers, in particular the right atrium 4. Symptoms depend on the degree of infiltration of the heart and surrounding structures.…”