Coexisting Benign and Malignant Tumours: A Cardiac Angiosarcoma Case Report

Abstract Background Primary cardiac tumours are rare. When patients present with both cardiac and extracardiac masses, clinicians may opt to biopsy accessible peripheral lesions to guide diagnosis, but benign findings can provide false reassurance. This case demonstrates the diagnostic pitfall of this approach when multiple concurrent pathologies coexist. Case Summary A 79-year-old male presented with progressive peripheral oedema, dyspnoea, and chest pain. Echocardiography revealed a 6.5 cm mobile intracardiac mass originating from the right atrial appendage. Pericardiocentesis drained 1100 cc of haemorrhagic fluid with negative cytology. PET-CT showed FDG uptake in the cardiac mass as well as multiple extracardiac lesions. Biopsy of a left upper arm lesion revealed a benign peripheral nerve sheath tumour. Photon-counting CT deemed angiosarcoma less likely due to limited contrast enhancement. These findings combined initially lowered suspicion for cardiac malignancy. The patient underwent surgical resection via median sternotomy. Histopathology revealed a high-grade angiosarcoma with positive resection margins (CD31+, CD34+, ERG+, Ki-67 40-60%). The postoperative course was uncomplicated, and the patient was transferred to the referring hospital on postoperative day four. Discussion This case demonstrates that benign pathology from peripheral lesions should not eliminate suspicion for cardiac malignancy when clinical features such as haemorrhagic pericardial effusion, right heart failure, and thromboembolic complications are present. Multiple concurrent pathologies can coexist, underlining the limitations of extrapolating peripheral biopsy results to cardiac masses.

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Publication Details

Journal
European Heart Journal - Case Reports
Published
2026-08-17
DOI
https://doi.org/10.1093/ehjcr/ytag591
Primary Topic
Cardiac tumors and thrombi
Type
article
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article

Coexisting Benign and Malignant Tumours: A Cardiac Angiosarcoma Case Report

Sulayman el Mathari, Jolanda Kluin, Loudy P. Priesterbach-Ackley, Jonathan R.G. Etnel et al.
European Heart Journal - Case Reports
Cardiac tumors and thrombi
article

Coexisting Benign and Malignant Tumours: A Cardiac Angiosarcoma Case Report

Sulayman el Mathari, Jolanda Kluin, Loudy P. Priesterbach-Ackley, Jonathan R.G. Etnel, Tiemen E T Holtrop
article en

Abstract

Abstract Background Primary cardiac tumours are rare. When patients present with both cardiac and extracardiac masses, clinicians may opt to biopsy accessible peripheral lesions to guide diagnosis, but benign findings can provide false reassurance. This case demonstrates the diagnostic pitfall of this approach when multiple concurrent pathologies coexist. Case Summary A 79-year-old male presented with progressive peripheral oedema, dyspnoea, and chest pain. Echocardiography revealed a 6.5 cm mobile intracardiac mass originating from the right atrial appendage. Pericardiocentesis drained 1100 cc of haemorrhagic fluid with negative cytology. PET-CT showed FDG uptake in the cardiac mass as well as multiple extracardiac lesions. Biopsy of a left upper arm lesion revealed a benign peripheral nerve sheath tumour. Photon-counting CT deemed angiosarcoma less likely due to limited contrast enhancement. These findings combined initially lowered suspicion for cardiac malignancy. The patient underwent surgical resection via median sternotomy. Histopathology revealed a high-grade angiosarcoma with positive resection margins (CD31+, CD34+, ERG+, Ki-67 40-60%). The postoperative course was uncomplicated, and the patient was transferred to the referring hospital on postoperative day four. Discussion This case demonstrates that benign pathology from peripheral lesions should not eliminate suspicion for cardiac malignancy when clinical features such as haemorrhagic pericardial effusion, right heart failure, and thromboembolic complications are present. Multiple concurrent pathologies can coexist, underlining the limitations of extrapolating peripheral biopsy results to cardiac masses.

European Heart Journal - Case Reports
Erasmus University Rotterdam (NL)
Good health and well-being
Openalex Percentile: Top 9%
Cardiac tumors and thrombi
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Coexisting Benign and Malignant Tumours: A Cardiac Angiosarcoma Case Report — Sulayman el Mathari, Jolanda Kluin, et al. · European Heart Journal - Case Reports (2026) | TGRS Research Map | TGRS