Primary cardiac lymphoma: B- and T-cell cases at a specialist UK centre

Abstract

Background: Primary cardiac lymphoma is a very rare malignancy, which is typically of a non-Hodgkin’s type, and involves only the heart and pericardium with no or minimal evidence of extracardiac involvement. In the past, they were frequently diagnosed at autopsy but modern imaging technology now permits early diagnosis and treatment which allows for improved prognosis.Patients and methods: This report describes the wide spectrum of clinical presentation, difficulty with correct clinical diagnosis, complications of treatment and pathologic findings of one of the largest series of primary cardiac lymphomas at a specialist UK centre. Our series comprised five males and one female with an age range of 10–81 years.Results: Most cases involved at least two chambers with the ventricles being the most common site. Clinical presentation included arrhythmias, valve incompetence, cardiac failure, pericardial effusion, embolic stroke and sudden death. Our study, in contrast to other series, included both B- and T-cell lymphomas.Conclusions: All six cases illustrate the wide spectrum of clinical presentation of lymphomas presenting primarily in the heart and emphasise that histology of all mass lesions is essential. Other series are small like ours highlighting the rarity of these tumours in the heart with the emphasis on imaging, early diagnosis and treatment.