Case 9 - Cardiac angiosarcoma

Summary

Imaging description

Cardiac angiosarcoma is an aggressive, infiltrative mass that is most often found in the wall of the right atrium. On CT, they are typically low-attenuation, broad-based lobulated masses with heterogenous enhancement post-contrast (Figure 9.1). On MRI, cardiac angiosarcoma is heterogenous due to presence of intralesional hemorrhage and necrosis; however, it is predominantly T2 hyperintense and T1 isointense to myocardium (Figure 9.2). Flow voids within the mass may be seen due to extensive vascularity. Occasionally, tumors may be avidly enhancing with post-contrast tumor attenuation at CT and MRI nearly identical to the blood pool (Figure 9.1). Tumor infiltration into adjacent structures such as the pericardium, superior or inferior vena cava, or interatrial septum is common (Figure 9.3). Hemorrhagic pericardial effusions are typically seen and can be large.

Importance

Cardiac angiosarcomas may be difficult to diagnose at enhanced chest CT due to their hypervascularity (Figure 9.1). They can appear very similar in attenuation to the blood pool and may be overlooked if close attention is not paid to the heart. Early, small tumors may be also difficult to appreciate on non-gated CT examinations due to motion. The presence of a de novo hemorrhagic pericardial effusion should prompt close inspection of all cardiac chambers for this entity. Accurate diagnosis is critical for proper patient management.

Typical clinical scenario

Cardiac angiosarcomas are rare, although they are the most common primary cardiac malignancy. They represent 10% of all primary cardiac tumors. Patients will most often present with chest pain, tamponade, or right heart failure. The majority of patients will have metastastic disease at usually survive beyond 12 months.

Differential diagnosis

Cardiac metatastases are much more common than primary cardiac angiosarcoma and should always be considered. These will commonly be multiple and will be associated with additional sites of metastastic disease throughout the body. Lung, melanoma, and breast cancer are common tumors to metastasize to the heart.