Primary tumors of the heart.

PURPOSE OF REVIEW: 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.

RECENT FINDINGS: 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.

SUMMARY: 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.