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Occult primary cardiac lymphomas causing unexpected/sudden death or acute heart failure

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Abstract

Three cases of unexpected/sudden death (N = 2) or acute heart failure (N = 1) were investigated in our centre. The first patient died unexpectedly after surgery for cardiac tamponade and constrictive pericarditis; at autopsy, gross features mimicked a pericardial mesothelioma. The second patient died suddenly after recovering from a respiratory insufficiency episode; autopsy revealed an epicardial mass encircling the right coronary artery. The third patient presenting symptoms mimicked a fulminant myocarditis and she underwent endomyocardial biopsy. In all cases, histology disclosed a diffuse large B cell non-Hodgkin lymphoma, localized to the pericardium together with the right ventricle and the conduction system, to the epicardium and the right coronary artery or to the myocardium, respectively. Histology was crucial for the diagnosis, the atypical presentation favouring other diagnostic hypotheses. Although primary cardiac lymphoma is uncommon and usually shows a sub-acute onset, it may also cause unexpected/sudden death or acute heart failure.

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Authors and Affiliations

Authors

Contributions

Andrea De Martino: collection of data, preparation of the manuscript, surgical procedures. Federico Del Re: collection of data, surgical procedures. Carlo Barzaghi: surgical procedures, data collection. Uberto Bortolotti: revision and analysis of data. Luigi Papi: autopsy procedure analyses. Angela Pucci: histology and immunohistochemistry analyses on endomyocardial biopsy, surgical and autopsy samples, writing and revision of the manuscript

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Correspondence to Angela Pucci.

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The study was conducted according to our Institutional Review Board and in adherence to the Declaration of Helsinki. A written informed consent was obtained from all patients or their closest relatives.

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The authors declare that they have no conflict of interest.

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This article is part of the Topical Collection on Quality in Pathology

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De Martino, A., Del Re, F., Barzaghi, C. et al. Occult primary cardiac lymphomas causing unexpected/sudden death or acute heart failure. Virchows Arch 477, 603–607 (2020). https://doi.org/10.1007/s00428-020-02819-3

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  • DOI: https://doi.org/10.1007/s00428-020-02819-3

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