Cardiac tumors: Updated classifications and main clinico-pathologic findings

This review article - which is devoted to a wide range of physicians, e.g., pathologists, clinicians, radiologists, and general practitioners - is an up-to-date clinico-pathological description of cardiac tumours. Although rare, cardiac tumours are key components in oncology practice since both their early diagnosis and appropriate management denote urgent needs. Primary cardiac tumours (PCTs) are categorized in recent WHO classifications as well as in other scientific contributions. Their incidence is around 0.02 % while their prevalence is between 0.001 % and 0.03 %. Among PCTs, benign lesions account for 85 % of cases, while malignant neoplasms for 15 %. Compared to PCTs, secondary cardiac tumours are 20-30-fold more common. The most frequent PCTs in adulthood are papillary fibroelastoma and cardiac myxoma, while, in childhood, cardiac rhabdomyoma. Heart metastases may occur through direct extension, or, indirectly, via bloodstream, lymphatics or intracavitary diffusion. Thoracic cancers (e.g. from pleura, lung, breast) are the most frequent cause of cardiac metastasis followed by oesophageal and haematological malignancies. Symptoms of PCTs (e.g. arrhythmias, dyspnoea, chest discomfort, syncope) are usually the result of both their regional involvement and location. Non-invasive imaging techniques (e.g. echocardiography, MRI, CT) and biopsy are key means in delineating tumour characteristics, size, and adjacent structure involvement. Most PCTs require surgery, which is recommended to prevent life-threatening complications. While many benign cardiac neoplasms may be completely resected, the treatment of choice for malignant PCTs and metastases is a combination of surgery, radio- and/or chemotherapy, as well as new alternative treatments, which may prolong survival in a small patient subset.