C14 Case Report: Multimodality Image–challenge in Primary Cardiac Diffuse Large B–cell Lymphoma

Abstract

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.