Occult primary cardiac lymphomas causing unexpected/sudden death or acute heart failure
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BRIEF REPORT
Occult primary cardiac lymphomas causing unexpected/sudden death or acute heart failure Andrea De Martino 1 & Federico Del Re 1 & Carlo Barzaghi 1 & Uberto Bortolotti 1 & Luigi Papi 2 & Angela Pucci 3 Received: 8 December 2019 / Revised: 4 April 2020 / Accepted: 16 April 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
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. Keywords Cardiac lymphoma . Sudden death . Endomyocardial biopsy . Histology
Introduction Primary cardiac lymphomas (PCL) are rare, accounting for 1– 2% of the surgically resected heart tumors, their incidence ranging between 0.15 and 1% at post-mortem examination [1, 2]. The clinical symptoms are nonspecific and mainly related to the tumor location whereas the prognosis largely depends upon early diagnosis and treatment [2, 3]. Although the modern imaging techniques together with the endomyocardial biopsy and the cardiac surgery evolution have dramatically increased in vivo diagnosis of cardiac masses, cardiac tumors may still cause acute heart failure or even sudden death [2–6]. We herein describe three cases of PCL in immunocompetent patients, presenting with unexpected/sudden death or acute
This article is part of the Topical Collection on Quality in Pathology * Angela Pucci [email protected] 1
Department of Cardiac Surgery, Pisa University Hospital, Pisa, Italy
2
Department of Forensic Science, University of Pisa, Pisa, Italy
3
Department of Histopathology, Pisa University Hospital, Pisa, Italy
heart failure, the signs and symptoms mimicking other disorders. Histology was crucial for the diagnosis, the clinical presentation favouring other diagnostic hypotheses.
Case report Case # 1 A 71-year-old man was referred to our Cardiac Surgery Department because of cardiac tamponade with atrial fibrillation. A pericardiocentesis, yielding 1200 cc of hematic fluid, resulted only in a temporary improvement and the patient underwent partial pericardiectomy that showed a severely and diffusely thickened pericar
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