Primary Cardiac Angiosarcoma: A Review

Abstract

Primary cardiac angiosarcoma is a rare and aggressive malignancy originating from the endothelial lining of cardiac blood vessels. This review covers various aspects of the disease, including its pathogenesis, clinical presentation, diagnosis, treatment, and prognosis. The primary characteristic of cardiac angiosarcoma is the rapid growth of abnormal blood vessels that invade the heart muscle, leading to the destruction of healthy tissue. Due to its infiltrative nature and early spread, diagnosing and treating cardiac angiosarcoma present significant challenges. Transesophageal echocardiography (TEE) plays a crucial role in diagnosing cardiac tumors such as angiosarcoma due to its high sensitivity. Additional imaging techniques such as computed tomography (CT) and cardiac magnetic resonance imaging (MRI) help assess tumor anatomy and identify metastases. Histopathological examination and immunohistochemistry are essential for confirming the diagnosis, as they reveal distinct histological features and specific endothelial markers associated with primary cardiac angiosarcoma. Targeted therapies directed at the angiogenic mechanisms and molecular abnormalities hold promise for improving treatment outcomes. Early detection of primary cardiac angiosarcoma remains challenging due to its rarity, and the prognosis is generally poor due to advanced disease at the time of diagnosis. The review emphasizes the importance of a multidisciplinary approach and collaboration among different specialties to optimize the diagnosis, treatment, and follow-up care of patients with primary cardiac angiosarcoma. The ultimate goal is to enhance diagnostic methods and therapeutic approaches by advancing knowledge and promoting further research into this aggressive malignancy.

Keywords: adult cardiac surgery; angiosarcoma; cardiology; primary cardiac angiosarcoma; tumor.

Figure 1. (a) Angiosarcoma moderately differentiated (coloring H&E, 10×). (b) Greater enlargement angiosarcoma moderately differentiated (coloring H&E, 40×). (c) Tumor cells were positive for CD31 (immunohistochemical investigation, 20×). (d) Tumor cells were positive for CD34 (immunohistochemical investigation, 20×). (e) Tumor cells were positive for FVIII rAg (immunohistochemical investigation, 20×). (f) Positive for Mib-1 60% (40×).

Image adapted from Antonuzzo et al. [6] FVIII rAg: factor VIII-related antigen