Primary cardiac angiosarcoma: modern methods of diagnosis and treatment

Abstract

The review is dedicated to such a rare disease as primary cardiac angiosarcoma (PCA). The following imaging methods are used for its diagnosing: transthoracic and transesophageal echocardiography, computed tomography, positron emission tomography and magnetic resonance imaging. To verify the diagnosis, a biopsy with cytological and immunohistochemical analysis is performed. The symptoms of PCA is non-specific and often manifests itself only in the late stages, which leads to a significant deterioration of prognosis. Treatment of PCA requires a multifaceted approach. It mainly involves the achievement of a curative resection (R0 resection). Regarding chemotherapy, neoadjuvant therapy with doxorubicin and ifosfamide is effective. In addition to these agents, cyclophosphamide, anthracycline, vincristine and others are used. Today, the use of targeted therapy with imatinib, sorafenib, pazopanib, and bevacizumab is promising. However, it requires further analysis. The article also describes other therapy methods using a beta-blockers and radiation exposure.