Primary tumors of the heart

Primary cardiac tumors are a rare disease, which may have severe clinical consequences. Malignant tumors may be misdiagnosed as mixomas, with improper treatment. The aim of this review is to report how to make a differential diagnosis using old and modern tools, the latest trends in tumor classification and treatment, and their possible impact on prognosis.

Papillary fibroelastomas seem to be more frequent than previously reported, and surgery is also suggested in asymptomatic patients. A genetic background has been identified for some myxomas and angiosarcomas. Malignant tumors are now classified as soft-tissue sarcomas of other organs. Immunohistochemistry and molecular diagnosis aid in recognizing several subtypes of sarcomas, leading to the possibility of targeted chemotherapy. The reports of single-center and multicenter experiences, collecting a large number of treated patients, analyzed the impact on prognosis of different approaches. The best results for survival and event-free survival are obtained with the multimodality approach.

Before referring a patient with cardiac tumor to the cardiac surgeon, a presumptive diagnosis of benignity or malignancy should be obtained. Malignant tumors should be referred to a cardiac tumor team with special expertise, in order to plan the best therapeutic approach.