Cardiac tumors can be broadly classified into those that arise primarily in the heart and those that reflect metastatic disease from a distant primary source. Primary cardiac tumors can be further divided into those that are pathologically benign and those that are malignant. Overall, primary cardiac tumors are relatively uncommon, whereas secondary involvement of the heart or pericardium occurs in as many as 20% of patients with end-stage metastatic cancer. While patients with cardiac tumors may present with a variety of symptoms, many patients are asymptomatic at the time of diagnosis as the tumor may be identified incidentally on imaging studies performed for other reasons. Such findings need to be differentiated from other cardiac masses such as vegetation, thrombus, or myocardial hypertrophy. Echocardiography is usually the initial method of evaluation of cardiac tumors; however, a variety of imaging modalities are now available and a multimodality approach is often necessary for accurate diagnosis and clarification of treatment options (Table 266-1).
TABLE 266-1Imaging Modalities and Their Utility in the Evaluation of Cardiac Tumors ||Download (.pdf) TABLE 266-1 Imaging Modalities and Their Utility in the Evaluation of Cardiac Tumors
|Modality ||Utility in Cardiac Tumor Evaluation |
|Transthoracic echocardiography (TTE) (including 2-D, 3-D, and contrast) ||Assessment of tumor location and size, and its impact on adjacent structures (e.g., valves, pericardium). |
|Transesophageal echocardiography (TEE) ||Improved tumor characterization and spatial resolution compared with TTE. May aid in determining surgical approach. |
|Cardiac MRI with gadolinium contrast ||Improved tissue characterization, definition of tumor size and identification of local invasion when compared with TTE or TEE. May differentiate tumor from thrombus. |
|Gated cardiac CT ||Provides anatomic assessment and tissue characterization of the tumor. Useful when patients cannot tolerate MRI or when MRI is not feasible (e.g., patients with implantable cardiac devices). Allows for better assessment of calcified lesions and evaluation of extra-cardiac tumor involvement. |
|Nuclear Imaging (including 18F-fluorodeoxyglucose positron emission tomography [FDG-PET]) ||Definition of extra-cardiac disease. May be useful in diagnosis of certain cardiac tumors (e.g., neuroendocrine tumors) but assessment of smaller tumors may be limited by surrounding myocardial FDG uptake. |
Primary tumors of the heart are rare. Approximately three-quarters are histologically benign, and the majority of these tumors are myxomas. Malignant tumors, almost all of which are sarcomas, account for 25% of primary cardiac tumors. All cardiac tumors, regardless of pathologic type, have the potential to cause life-threatening complications. Many tumors are now surgically curable; thus, early diagnosis is imperative.
Cardiac tumors may present with a wide array of cardiac and noncardiac manifestations. These manifestations, which depend in large part on the location and size of the tumor as well as its impact on surrounding cardiac structures, are often nonspecific features of more common forms of heart disease, such as chest pain, syncope, congestive heart failure (CHF), ...