Primary cardiac lymphoma

Abstract

Primary cardiac lymphoma (PCL) is a rare neoplasm involving the heart, pericardium or both. Patients with PCL have a median survival of approximately 7 months based on literature. We report a unique case of a 63-year-old woman presenting with worsening exertional dyspnoea and symptoms of superior vena cava obstruction. Investigations revealed a large right atrial (RA) tumour with obstructive features. In the past, PCL was frequently diagnosed at postmortem but with modern imaging and biopsy techniques, this allows early diagnosis and treatment of this rapidly fatal disease. Our patient had a trans-jugular venous biopsy of the RA mass under trans-oesophageal echocardiography guidance. This confirmed the diagnosis of PCL. She subsequently completed seven cycles of chemotherapy and is currently in remission 20 months after diagnosis.

A rare primary pericardial DLBCL masquerading as an unexplained malignant pleural effusion in an elderly woman: A case report

“…Cardiac and pericardial lymphomas involvement is extremely rare, accounting for 0.5% of cardiac involvement and 1% of extranodal NHL. It is predominant in male patients in their 60s [ 6 , 15 ]. However, our patient was an elderly woman.…”

“…PCLs affect the right heart with a strong predilection for the right atrium, and only a few cases of lymphomas with the main bulk at the level of the left sections are described in the literature [ 15 ]. Some PCLs are common in immunocompromised patients with acquired immunodeficiency syndrome and transplant recipients receiving immunosuppressive therapy [ 1 , 14 , 15 ].…”

“…PCLs affect the right heart with a strong predilection for the right atrium, and only a few cases of lymphomas with the main bulk at the level of the left sections are described in the literature [ 15 ]. Some PCLs are common in immunocompromised patients with acquired immunodeficiency syndrome and transplant recipients receiving immunosuppressive therapy [ 1 , 14 , 15 ]. Previously, investigators also described HIV-negative patients with a B-cell lineage primary effusion lymphoma involving the pleural cavity, whose tumour cells lack HHV8 infection [ 1 , 16 ], as in our case.…”

Left-sided primary cardiac lymphoma: a case report

“…Primary cardiac lymphoma (PCL) is a rare entity accounting for less than 2% of primary cardiac tumors: it lacks a univocal definition, and the evidence in literature derives almost exclusively from case reports [1][2][3][4][5][6][7][8][9][10][11][12][13].…”

“…The diagnostic criteria for PCL have progressively broadened over time. The definition of PCL was initially reserved for a mass with exclusive involvement of the heart or pericardium, while at present, it can be made when the primary bulk of the lymphoma is localized in the heart or pericardium even if limited extracardiac lymphoma is present-mostly when the clinical manifestations are related primarily to cardiac dysfunction [2,3].…”