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

Our plain-English summary of Occult primary cardiac lymphomas causing unexpected/sudden death or acute heart failure, by A. De Martino, F. Del Re, C. Barzaghi, et al (Virchows Archiv, 2020).

The study

• Authors: A. De Martino, F. Del Re, C. Barzaghi, U. Bortolotti, L. Papi, A. Pucci
• Journal: Virchows Archiv
• Published: 2020;477(4):603-607
• Type of article: Case report
• Cited by: 4 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

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.

In plain English

This brief report from Pisa University Hospital in Italy described three patients with primary cardiac lymphoma, a cancer of immune cells that starts in the heart. The article does not discuss asbestos or any patient's exposure history. Its only link to mesothelioma is that one case looked like a pericardial mesothelioma before microscopic examination. A 71-year-old man died hours after surgery to remove part of a thickened pericardium, the sac around the heart. At autopsy the pericardium showed plaque-like thickening "highly suggestive" of pericardial mesothelioma. Microscopic examination instead showed diffuse large B cell non-Hodgkin lymphoma, which had also spread into the right ventricle and the heart's conduction system. A 77-year-old woman died suddenly in an ambulance. Autopsy found an 8 by 3 cm lymphoma mass encircling and compressing her right coronary artery. A 55-year-old woman suspected of fulminant myocarditis had a heart biopsy that showed lymphoma. After chemotherapy she had no evidence of residual or recurrent disease at 12 months. The authors concluded that the unusual presentations pointed toward other diagnoses and that microscopic examination was crucial for diagnosis.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Brief report describing three cases (case series)
• Who was studied: Three immunocompetent patients seen at Pisa University Hospital, Italy: a 71-year-old man, a 77-year-old woman with COPD and high blood pressure, and a previously healthy 55-year-old woman. Dates of the cases are not given.
• Main results:
  • Case 1 (71-year-old man): at autopsy, the pericardium showed plaque-like thickening that looked like pericardial mesothelioma and appeared to infiltrate the right ventricle wall.
  • Case 1: microscopic examination and immune-marker testing showed a diffuse large B cell non-Hodgkin lymphoma, not mesothelioma. It involved the atrioventricular node region and the right bundle branch of the conduction system.
  • Case 2 (77-year-old woman who died suddenly): autopsy found an epicardial mass measuring 8 by 3 cm encircling and compressing the proximal right coronary artery. It was a diffuse large B cell lymphoma.
  • Case 2: the lymphoma did not involve the conduction tissue or any other cardiac structure, organ or tissue.
  • Case 3 (55-year-old woman suspected of fulminant myocarditis): a lymphoid infiltrate was found in two of four endomyocardial biopsy samples. A diffuse large B cell non-Hodgkin lymphoma was diagnosed.
  • Case 3: after R-CHOP chemotherapy, the patient was recovering well at 12 months with no evidence of residual or recurrent disease.
• The authors' conclusion: The authors concluded that an atypical presentation favouring other diagnoses characterized all three cases. They found microscopic examination crucial for diagnosis, whether on heart biopsy or after death. In their view, early diagnosis by heart biopsy positively influenced the outcome in the case that mimicked fulminant myocarditis.

Topics

Medical terms in this study, explained in our Lexicon: Cardiac Tamponade, Autopsy, Pericardial Mesothelioma, Biopsy, Histology, Pericardium.

Related studies

• Cisplatin-induced sudden cardiac death with hemodynamic collapse: a severe adverse drug reaction: Case report — S. Abramavicius, M. Zemaitis, et al. (2017)
• Primary tumors and cysts of the heart and pericardium — H.A. McAllister (1979)
• Diffuse pleural thickening following heart failure-related pleural effusions in an asbestos exposed patient — M. Evison, P. Barber (2015)
• Cardiac masses: assessment by MR imaging — J.T. Lund, R.L. Ehman, et al. (1989)
• Primary mesothelioma of atrioventricular node: cause of sudden death — E.H. Hemsted (1986)
• Natriuretic peptides in pleural effusions: Beyond a diagnosis of heart failure — J.M. Porcel (2020)

Browse more research in Virchows Archiv, or return to medical research.