A 76-year-old Caucasian woman presented to our hospital with progressive exertional fatigue and weight-loss over 2 months.Her past medical history revealed a recent local-hospital admission because of atrioventricular third-degree block, requiring permanent pacemaker implantation. At presentation her cardiovascular exam was normal.Laboratory studies revealed elevated LDH and PCR; electrolytes, cardiac biomarkers and complete blood count were normal. ECG demonstrated AF and ventricular stimulation.Transtoracich echocardiography showed LV hypertrophy in the basal segments, with normal ejection fraction and a reduced LV-GLS with a relative apical sparing pattern.There was a large, well-circumscribed mass arising from the right atrium, with a contextual moderate pericardial effusion (Fig1).Vasculitic, thrombophilic and infectious work-up were negative.The patient was immunocompetent.
CMR was proposed but could not be performed due to patient claustrophobia. ECG gated cardiac CT was carried out for a better differential diagnosis of the mass, as well as to look for possible embolization or primary tumours, in the hypothesis of cardiac metastases. A CT total body showed a large ill-defined mass invading the interatrial and interventricular septum with projections into the left ventricle. Not enlarged lymph nodes were detected with neither hepatosplenomegaly nor abdominal lymphadenopathy. A total body 18FDG PET revealed significant tracer uptake in the heart and pericardium without extracardiac uptake, consistent with a malignant cardiac mass (Fig2).LV endomyocardial biopsy was attempted: pathology revealed large B-cell lymphoma expressing CD20, Bcl-2, Bcl-6, Mum-1, C-myc and with a high Ki67 proliferation rate.
The patient underwent six cycles of chemotherapy(R-POCH).Echocardiography imaging after the second cycle showed significant reduction in myocardial wall thickness and concomitant improvement in LV-GLS. Repeated PET–CT scans 6 months post-chemotherapy showed complete remission with no evidence of FDG-avid lymphoma (Fig3).
Primary cardiac lymphoma (PCL) is an extranodal non-Hodgkin’s B-cell lymphoma exclusively located in the heart or pericardium, accounting for 1-2% of primary cardiac tumours. PCL often presents with non-specific clinical symptoms making the diagnosis challenging. Multimodality approach and myocardial biopsy are essential in the diagnostic work-up. Early diagnosis and treatment are crucial since a good response to chemotherapy is expected