Abstract

Abstract Introduction Primary cardiac lymphomas in immunocompetent patients are an uncommon malignancy, accounting for 1.3% of primary cardiac tumors, while metastases secondaries to extracardiac lymphomas are more common. Mean age at diagnosis is 63 years old. They mainly involve the right side of the heart, particularly right atrium. Symptoms of primary cardiac lymphoma are non-specific. Cardiac lymphoma typically follows an aggressive clinical course and the prognosis is poor, with more than 60% of patients dying within 2 months of diagnosis. Predictors of worse outcome are immune status, left ventricular involvement, arrhythmias and the presence of an extracardiac disease. Case report 48-year-old obese and hypertensive man, in therapy for OSAS. In February 2022 admission to the emergency department due to jugular pressure and mild exertion dyspnoea. Neck CT excluded inflammatory collections; echocardiography showed normal cavitary dimensions and biventricular systolic function, absence of major valvulopathies and circumferential pericardial effusion of n.d.d. with TD max 2.5 cm and partial collapse of the right atrium, for which started corticosteroid therapy. Chest CT showed mediastinal lymphadenopathy and persistence of pericardial effusion. In April, due to worsening dyspnoea, cardiac CT demonstated a solid mass at the passage from the superior vena cava to the right atrium (50×37×67 mm), leading to compression-infiltration phenomena on the superior vena cava, and thrombosis of the right mammary vein. Cardiac MR confirmed a voluminous expansive-infiltrative lesion, with an irregular lobular profile, projecting into the superior vena cava and extended to the right atrium. This mass appears very iperintensive in T2 stir sequences with an early contrast impregnation and uneven intralesional late enhancement (picture 1). With suspicion of lymphoma, after collegial discussion between thoracic surgeons, cardiologists, interventional radiologists and cardiac surgeons, a right transjugular radio- and echo-guided biopsy was performed to complete diagnostic iter; biopsy was positive for peripheral B lymphocyte-derived large-cell diffuse lymphoma with large necrotic and subnecrotic areas. Chemotherapy showed initial benefit,with a clear reduction in size of the cardiac mass and mediastinal lymphnodes at 2 months follow up, with regression of dyspnoea. Conclusions In our patient, despite an early lymphoma onset and a typical poor prognosis compared to literature, an early diagnostic-therapeutic iter, an immunocompetent status, the absence of extracardiac disease and a preserved left ventricular ejection fraction, ensured an immediate clinical benefit with an improvement of his quality of life.

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