Neoplasms of the heart can be divided into primary cardiac tumors arising in the heart and secondary cardiac tumors from metastasis. Primary cardiac tumors can be further stratified into benign and malignant tumors. Between 10 and 20% of patients dying of disseminated cancer have metastatic involvement of the heart or pericardium.1,2 Surgical resection is seldom possible or advisable for these tumors, and intervention usually is limited to drainage of malignant pericardial effusions and/or diagnostic biopsies.
The incidence of primary cardiac neoplasm ranges between 0.17 and 0.19% in unselected autopsy series.3-5 Seventy-five percent of primary cardiac tumors are benign, and 25% are malignant.2,6 Fifty percent of the benign tumors are myxomas, and 75% of malignant tumors are sarcomas.2,6 The clinical incidence of these tumors is 1 in 500 cardiac surgical patients. With the exception of myxomas, most surgeons will rarely encounter primary cardiac tumors. The purpose of this chapter is to summarize useful information for the evaluation and management of patients with cardiac tumors and to provide a reference for additional study.
A primary cardiac neoplasm was first described by Realdo Colombo in 1559.7 Alden Allen Burns of Edinburgh described a cardiac neoplasm and suggested valvular obstruction by an atrial tumor in 1809.8 A series of six atrial tumors, with characteristics we now recognize as myxoma, was published in 1845 by King.9 In 1931, Yates reported nine cases of primary cardiac tumor and established a classification system similar to what we use today.10 The first antemortem diagnosis of a cardiac tumor was made in 1934 when Barnes diagnosed a cardiac sarcoma using electrocardiography and biopsy of a metastatic lymph node.11 In 1936, Beck successfully resected a teratoma external to the right ventricle,12 and Mauer removed a left ventricular lipoma in 1951.13 Treatment of cardiac tumors was profoundly influenced by two events: the introduction of cardiopulmonary bypass in 1953 by John Gibbon, which allowed a safe and reproducible approach to the cardiac chambers, and the introduction of cardiac echocardiography, which allowed safe and noninvasive diagnosis of an intracardiac mass. The first echocardiographic diagnosis of an intracardiac tumor was made in 1959.14 An intracardiac myxoma was diagnosed by angiography in 1952 by Goldberg, but attempts at surgical removal were unsuccessful.9 A large right atrial myxoma was removed by Bhanson in 1952 using caval inflow occlusion, but the patient died 24 days later.15 Crafoord in Sweden first successfully removed a left atrial myxoma in 1954 using cardiopulmonary bypass,16 and Kay in Los Angeles first removed a left ventricular myxoma in 1959.17 By 1964, 60 atrial myxomas had been removed successfully, with improved results owing to the increasing safety of cardiopulmonary bypass and use of echocardiography for detection. Operations are currently performed routinely on patients with atrial myxoma with minimal mortality.6,18–21 Primary malignant tumors, however, continue to represent a challenge.