Diffuse large B cell lymphoma associated with chronic inflammation arising within atrial myxoma: aggressive histological features but indolent clinical behaviour

Aims Primary cardiac lymphoma (PCL) is a rare neoplasm. PCL is fatal, unless it is diagnosed and treated early. Recently, a small number of cases of diffuse large B cell lymphoma (DLBCL) arising within atrial myxoma have been reported in immunocompetent patients and showed aggressive histologic features but an indolent clinical behaviour. Methods And Results We presented four unusual cases of Epstein-Barr virus (EBV)-positive DLBCL arising within atrial myxoma with detailed clinical, histologic, immunophenotypic and genotypic features in immunocompetent patients, and reviewed the literature for 11 similar cases. All the patients appeared to have morphologic features of DLBCL, B-lineage immunophenotype, high proliferative index and latency type III of EBV infection. They achieved complete tumour resection without chemotherapy or radiotherapy after surgery and were healthy at 10-year, 7-year, 7-month and 3-month follow-ups, respectively. Conclusions We suggest that this lymphoma should be noted as a unique DLBCL associated with chronic inflammation (DLBCL-CI) because of an indolent clinical behaviour to avoid excessive or unnecessary treatments. In addition, early accurate diagnosis and complete resection of this tumour are crucial for the patients’ medical prognosis. This article is protected by copyright. All rights reserved.