Introduction: While primary malignant tumors of the heart are rare, angiosarcomas are the most common cardiac malignant tumors.

Case Presentation: We describe a 23-year-old woman who presented with a right atrial mass, which was discovered to be a cardiac angiosarcoma. We demonstrate the use of several noninvasive imaging modalities along with pathology confirmation for the definitive and comprehensive diagnosis of a cardiac angiosarcoma, a rare entity by itself.

Conclusions: With the increasing availability of noninvasive imaging techniques, the diagnosis of angiosarcomas can be made at earlier stages. If angiosarcomas are left untreated, their prognosis is very poor. Therapeutic options include surgical excision, chemotherapy, radiation therapy, and heart transplantation or a combination of these.

Primary tumors of the heart, the majority of which are benign, are rare with an incidence at autopsy ranging from 0.0017% to 0.02% (1, 2). The most common cardiac malignancies are sarcomas, and angiosarcomas account for the majority of these malignancies in the adult population, as in the present case. These are malignant neoplasms that more commonly affect the right side of the heart, are highly invasive to surrounding tissue, and have a high potential to metastasize.

A 23-year-old asymptomatic woman had a screening transthoracic echocardiogram because of a family history of cardiomyopathy and was found to have a mass in the right atrium (RA) and moderate pericardial effusion. Further evaluation by transesophageal echocardiography showed that the mass was attached to the lateral wall of the RA, with partial obstruction of the tricuspid valve, producing a mean diastolic pressure gradient of 4 mmHg (Figure 1). As is shown in Figure 2, cardiac magnetic resonance imaging demonstrated attachment of the mass to the lateral, superior, anterior, inferior, and posterior walls of the RA. There was partial obstruction of the coronary sinus and invasion into the pericardial space, with a moderately large pericardial effusion. Following gadolinium contrast (Gadodamide, Omniscan, Amersham, Piscataway, NJ), there was slow heterogeneous enhancement of the mass (Figure 2D, 2E, and 2F). Fluorodeoxyglucose-positive emission tomography (FDG-PET, Figure 3) did not show metastatic disease and displaced heterogeneous intense hypermetabolism, consistent with a tumor tissue. The patient underwent successful surgical excision of the RA mass (Figure 4A) along with RA and coronary sinus reconstruction with an autologous pericardial patch, creation of right pulmonary window, and drainage of the pericardial effusion. Histologic examination (Figure 4B) confirmed that the mass was a high-grade angiosarcoma, with positive margins along the right atrial free wall.

Patients with angiosarcomas usually present with non-specific symptoms and as such these are most often found incidentally or at autopsy. Pericardial effusion is a common finding, although fluid cytology has a low diagnostic yield (2, 3). With the increasing availability of noninvasive imaging techniques, diagnosis can be made at earlier stages. If left untreated, angiosarcomas have a poor prognosis. Therapeutic options include surgical excision, chemotherapy, radiation therapy, and heart transplantation or a combination of these (2). The reported response to therapy is variable: some authors have reported only a modest response to therapy and a survival of only 6-9 months (2), and the longest survival was 53 months reported in a single case (4).