Abstract
Cardiac tumours are rare. The majority are metastatic from elsewhere, with primary cardiac tumours representing only 0.02% of tumours in autopsy series. Their prevalence in the general population is difficult to estimate. Malignancies associated with metastasis to the heart include melanoma, carcinomas (most commonly of the lung and breast), sarcomas and lymphomas.
The clinical presentation of cardiac tumours is varied and is usually dependent upon the site and size of the lesion, rate of growth and invasiveness, and any associated complications such as pericardial effusion, conduction disturbances, cardiac failure and systemic or pulmonary emboli. Non-specific constitutional symptoms such as fever, malaise and weight loss may also be encountered.
With the exception of lymphoma, the treatment of choice for cardiac tumours is complete resection in combination with chemotherapy or radiotherapy, although targeted therapies may provide future therapeutic options. Some cardiac tumours are part of genetic syndromes or are associated with a genetic predisposition and therefore have implications for genetic counselling.
Over 75–90% of primary cardiac tumours are benign, and the prevalence of each histological type of primary cardiac tumour is age-related. Cardiac myxomas are the most common benign cardiac neoplasms, although due to better imaging techniques papillary fibroelastomas may be more frequent than previously thought. Other benign lesions of the heart include rhabdomyoma, cardiac fibroma, cystic tumour of the atrioventricular node, cardiac lipoma and haemangioma. The heart may rarely be involved by inflammatory myofibroblastic tumour. Other primary cardiac entities include histiocytoid cardiomyopathy and lipomatous hypertrophy.
Most malignant cardiac tumours are sarcomas, comprising rhabdomyosarcoma, which predominates in children, angiosarcoma (representing 40% of cardiac sarcomas) and other types of sarcoma. Up to 2% of malignant cardiac lesions represent non-Hodgkin lymphoma.
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Rassl, D.M. (2019). Cardiac Tumours. In: Suvarna, S. (eds) Cardiac Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-24560-3_12
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