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Learning objectives
To understand the differential diagnosis of cardiac tumours including non-neoplastic masses and normal cardiac structures.
To review the definitions, classification and aetiology of cardiac tumours.
To appreciate the clinical presentation, diagnostic workup and imaging findings of cardiac tumours.
To learn the general principles for the contemporary management of cardiac tumours, including indication for cardiac surgery, chemotherapy and radiotherapy.
Introduction
Cardiac masses include tumours, thrombi, vegetations, calcific lesions and other rare conditions. Unlike most other organs, the malignant behaviour in the heart has haemodynamic consequences due to blood flow obstruction, embolism and electrical or mechanical dysfunction.1–3 After a presentation of the various categories of cardiac masses, we will focus on the diagnostic workup, where the clinical setting often provides the key for interpretation. Finally, management of cardiac tumours will be discussed.
Types of cardiac mass
Cardiac tumours
These include benign tumours and tumour-like lesions, malignant tumours and pericardial tumours (table 1).4 WHO has recently updated the classification of cardiac tumours; the major changes are removal of the term ‘malignant fibrous histiocytoma’, as synonymous with undifferentiated pleomorphic sarcoma; the incorporation of epithelioid haemangioendothelioma as an angiosarcoma with low-grade malignancy; the re-introduction of the osteosarcoma and myxofibrosarcoma subtypes; and the expansion of the molecular characterisation of many cardiac tumours.
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WHO classification of primary tumours of the heart
The estimated prevalence for primary cardiac tumours is 1:2.000 and for secondary tumours 1:100 autopsies, with a secondary/primary ratio of 20:1.1 ,2 The incidence of cardiac metastases ranges from 2.3% to 18.3% of patients with extracardiac malignancies (table 2).5
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Cardiac metastases in consecutive autopsies of patients with malignancies
Approximately 10% of primary cardiac tumours are malignant and 90% benign1–4 (figure 1). Among the benign cardiac tumours, the majority are myxomas, followed by papillary fibroelastomas, which are increasingly detected by echocardiography.6 ,7 The most …
Footnotes
Contributors CB is in charge of the cardiac tumour registry at the University of Padua and wrote the paper. SR, MV and GT are involved in the registry of cardiac tumours at our university and critically reviewed the paper.
Funding The authors are supported by the Registry for Cardio-cerebro-vascular Pathology, Veneto Region, Venice, Italy.
Competing interests None declared.
Provenance and peer review Commissioned; externally peer reviewed.