ESSENTIALS OF DIAGNOSIS
A rare but critical diagnosis in the evaluation of cardiac masses.
Although confirmation requires tissue biopsy, history, physical examination, and imaging characteristics can yield valuable diagnostic clues.
Cardiac tumors, although considered a rare diagnostic entity, provide a unique insight into the anatomic and pathophysiologic functioning of the heart. Reminiscent of benign, malignant, and metastatic neoplasms elsewhere in the body, theses tumors herald their presence by the secondary effects produced while enlarging within the confines of cardiac chambers and tissues. Because they rarely cause symptoms, cardiac tumors are generally unsuspected and may be discovered incidentally during evaluation of an unconnected condition. This chapter will help guide the astute clinician, focusing on when to consider this diagnosis and how to obtain the appropriate cardiovascular imaging studies. There are a wide variety of common cardiac tumors (Table 32–1).
Table 32–1.Classification of Common Cardiac Tumors |Favorite Table|Download (.pdf) Table 32–1. Classification of Common Cardiac Tumors
|Primary Cardiac Tumors |
Hamartoma (oncocytic cardiomyopathy)
Sarcoma: includes angiosarcoma, rhabdomyosarcoma, fibrosarcoma, osteosarcoma, leiomyosarcoma, liposarcoma, synovial sarcoma
Malignant fibrous histiocytoma
Benign or Malignant Tumors
Mesothelioma (includes cystic tumor of the atrioventricular node)
|Secondary Cardiac Tumors |
| Metastasis to the heart and pericardium: commonly from malignant melanoma or neoplasms of the lung, hematopoietic system, gastrointestinal tract, kidney, breast, thyroid, and soft tissue |
Primary tumors of the heart are rare, with a prevalence of 0.02% reported in combined data from multiple large autopsy series. Although cardiac myxoma has been traditionally reported as the most frequent tumor type in adults, increasing utilization of imaging studies have revealed a higher frequency of papillary fibroelastomas than previously known. In the pediatric population, rhabdomyomas represent the most common type of primary cardiac tumor (Table 32–2).
Table 32–2.Primary Cardiac Tumors: Location and Features |Favorite Table|Download (.pdf) Table 32–2. Primary Cardiac Tumors: Location and Features
|Cardiac Tumor ||Typical Location ||Interesting Features/Associations |
|Myxoma ||Left atrium ||Carney complex; constitutional symptoms |
|Papillary fibroelastoma ||Cardiac valves ||Can present with sudden death from coronary ostial obstruction |
|Fibroma ||Left ventricular myocardium ||Very large tumors that exert a mass effect |
|Rhabdomyoma ||Cardiac chambers ||Tuberous sclerosis |
|Angiosarcoma ||Right atrium ||Highly aggressive tumor |
|Rhabdomyosarcoma ||Cardiac chambers ||Commonest pediatric malignancy |
|Fibrosarcoma ||Left atrium ||White fish flesh tumor |
|Leiomyosarcoma ||Smooth muscle cells of pulmonary veins and arteries ||Epstein-Barr virus in immunosuppressed patients |
|Lymphoma ||Right heart chambers ||Immunocompromised patients |
|Paraganglioma ||Pericardium; left atrial wall ||Majority are hormonally inactive |
|Teratoma ||Pericardium ||Contain elements from all three germ cell layers |
The overwhelming majority of cardiac tumors are benign, ranging in incidence from 75 to 95%. Timely diagnosis is ...