Introduction
Background
Cardiac tumors may be primary or secondary, may be related to the heart muscle or pericardium, or may be direct extensions of primary tumors or metastases from adjacent structures.
The presence of a cardiac tumor upon clinical examination and electrocardiography was first documented in 1934. Until that time, cardiac tumors were only identified postmortem. Angiography was first used to demonstrate an intracavitary cardiac tumor in 1951.1
In general, primary cardiac tumors are of mesothelial or epithelial origin. Tumors include myxoma (see Images 1-13), fibroma, lipoma (see Image 14), rhabdomyoma, plasma cell granuloma, sarcoma, lymphoma (see Image 15), thymoma, hemangiopericytoma (see Images 16-19), fibroelastoma (see Images 20-21), angioma, hemangioma, angiomyolipoma/hamartoma, lymphangioma, and mycosis fungoides.2,3,4,5,6,7,8,9,10,11,12
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Endodermal tumors
Atrioventricular (AV) nodal tumors may contain neuroendocrine cells and, thus, may be of endodermal origin (as in polycystic tumor of the AV node or congenital endodermal heterotopia of the AV node).13,14 Cardiac paragangliomas have also been described.15,16 Patients with these tumors present with hypertension and elevated urinary catecholamine levels.17,18,19 These tumors tend to be left sided. Intracardiac pheochromocytomas also occur.20,21
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Pheochromocytoma
Myxomas
Myxomas are usually found in the left heart but can also occur in the right heart.22 Myxomas tend to be solitary and occur more often in women than in men. They are characteristically attached to the interatrial septum adjacent to the edge of the fossa ovalis (in approximately 85% of patients).23 Myxomas can invade the interatrial septum and the opposite atrium.24 Tumors may be pedunculated (see Images 10-13) or polypoid (see Images 3-5).23,25
Firm, oval masses are associated with dyspnea and show small vessels, tortuous vascularity, hemorrhage, and fibrosis. Soft papillary tumors are often associated with neurologic symptoms and brain infarcts.26 A study by Burke and Virmani showed that 41% of patients with myxomas have a surface thrombus and 41% have fibrosis.27 Patients with left-heart myxomas may present with mitral valve obstruction (see Images 1-2 and Images 10-13) or with findings suggestive of subacute bacterial endocarditis. Patients with right-heart myxomas may present with tricuspid valve disease, pulmonary embolism, or pulmonary hypertension.22,28,29
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Atrial Myxoma
Sarcomas
Primary cardiac sarcomas encompass a broad spectrum of tumors, such as angiosarcoma (see Image 22), osteosarcoma, fibrosarcoma, malignant fibrous histiocytoma, leiomyosarcoma (see Images 23-24), myxosarcoma, synovial sarcoma, neurofibrosarcoma, lymphosarcoma, reticulum cell sarcoma, and undifferentiated sarcoma.11,30,31,32,33,34,35,36,37,38,39 Angiosarcomas tend to be located in the right heart (see Image 22), and osteosarcomas tend to be found in the left heart.38,39 Primary cardiac liposarcoma has also been reported, though it is extremely rare. It is part of the differential diagnosis for a mass with fat signal intensity on magnetic resonance imaging (MRI) scans. Most liposarcomas involving the heart either arise from the pericardium or are metastatic. All are rare.12,40,41
Patients with cardiac tumors range in age at presentation from 1 to 75 years and may present with symptoms secondary to distal metastases.38,42,43,44 Osteosarcomas tend to calcify and are usually found in the left atrium.39 Angiosarcomas are the most common malignant primary cardiac tumor, and leiomyosarcomas are the second most common. Leiomyosarcoma frequently invades the mitral valve and pulmonary veins, and it frequently involves the left atrium.39 Other common sarcomas include rhabdomyosarcomas and fibrosarcomas. Fibrosarcoma is often necrotic and favors the left atrium.38,42,43,44
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Metastatic disease
Metastatic disease may result from contiguous extension, lymphangitic spread, or hematogenous spread.45 Many of the metastatic cardiac tumors are bronchogenic carcinomas (see Images 25-26), breast carcinomas, lymphomas, leukemia, carcinoid tumors, or melanomas.11,46,47 Metastatic cervical carcinoma has also been reported.48 Metastases to the heart tend to involve the myocardium rather than the valves or the endocardium.23
Alternatively, contiguous extension of a tumor may originate from primary or metastatic disease. For example, metastases to the lung can invade the mediastinum by means of local extension, such as by a malignant thymoma.49,50 Liver cancer, such as hepatocellular carcinoma, can extend cephalad via the inferior vena cava into the heart.51
Other abdominal tumors can affect the heart. Extension of a tumor thrombus via the inferior vena cava into the right atrium is a well-recognized complication of advanced renal cell carcinoma (see Image 27); this can also occur with cervical carcinoma and renal angiomyolipomas.52,53,54 Benign uterine leiomyomatosis can affect the heart intravenously.55,56,57 Germ cell tumors (eg, testicular teratomas), embryonal cell carcinomas, and choriocarcinomas can metastasize to the heart58,59,60 ; thyroid metastases have been identified61 ; and musculoskeletal tumors, such as hemangiopericytomas, can metastasize and grow in the heart.62
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Metastatic Carcinoma
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Pericardial tumors
Primary pericardial tumors include malignant spindle cell tumors, localized fibrous tumors (also called localized fibrous mesothelioma), pericardial cysts (see Images 28-30), liposarcomas, lipomas (see Image 31), and teratomas.39,63,64,65,66,67,68,69 Cardiac angiosarcomas tend to involve the pericardium secondarily and to produce hemorrhagic pericardial effusions.70
Pericardial mesotheliomas are frequently associated with asbestos exposure. Most specimens have identifiable asbestos fibers, particularly crocidolite and amosite fibers, as well as chrysotile (to a lesser extent).71 Not all mesotheliomas, however, arise in patients with a prior history of asbestos exposure.65,72 Lymphomas are frequently found in patients who are immunocompromised, and they frequently involve the pericardium.39 Liposarcomas are rare tumors that tend to be extracardiac and form large infiltrating masses.39
Cardiac valve tumors
Primary tumors of the cardiac valves and chordae are uncommon; however, when they do occur, they are usually fibroelastomas.73,74,75,76 Fibroelastomas are small tumors that may be an incidental finding at autopsy (see Images 20-21). They can be mistaken for heart valve vegetation. Lipomatous tumors of the heart valves also have been described.77
Cardiac tumors in childhood
Primary cardiac tumors that present in children include rhabdomyomas, intrapericardial teratomas, myxomas, fibromas, hemangiomas, mesotheliomas, multicystic hamartomas, epicardial lipomas, and rhabdomyosarcomas78,79 ; some tumors cannot be identified pathologically.80,81,82,83 Rhabdomyomas are the most common pediatric cardiac tumors. Most children with rhabdomyomas also have tuberous sclerosis,83 and these tumors can affect all 4 cardiac chambers.84 They frequently resolve spontaneously.42 Most primary pediatric tumors are diagnosed in the first year of life83 ; rhabdomyosarcoma is the most common pediatric malignant cardiac primary neoplasm. Compared with other primary cardiac sarcomas, rhabdomyosarcomas are more likely to involve the valves.39
Primary pediatric cardiac tumors present as murmurs and arrhythmias.83 Intrapericardial tumors can cause airway compression and subsequent dyspnea. In utero, fetuses can present with abnormal sonogram findings.85 These tumors can cause conduction abnormalities that can be evaluated by electrophysiologic testing and may be amenable to radiofrequency ablation.83
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Other cardiac masses
Some cardiac masses may not be tumors. Nonneoplasms or pseudotumors include thrombi or other variants of cardiac anatomy, such as a prominent pulmonary vein orifice (see Images 32-33), a right atrial Chiari network or crista terminalis (see Images 34-35), valvular vegetation, thrombi within a left ventricular aneurysm, lipomatous hypertrophy of the interatrial septum (see Image 36), rheumatoid nodules, pulmonary collapse, a ruptured chordae tendineae, intracardiac varices, tumors outside the heart and pericardium (which can resemble cardiac masses such as phrenic nerve tumors), and anatomic structures such as hiatal hernias (see Image 37).86,87,88,89,90,91
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Hiatal Hernia
Frequency
United States
The frequency of cardiac tumors is dependent on the frequency of autopsies performed in a specific region and the patient population being evaluated (see also Age).
- Benign primary tumors are more frequent than malignant primary tumors. In 2 autopsy series, the cumulative prevalence of primary cardiac tumors was reported as 0.001-0.3%.92,93 Overall, myxomas account for approximately 30% of all primary cardiac neoplasms. Ninety percent of atrial myxomas occur on the left, and 90% are solitary.6 Angiosarcomas account for 33% of all malignant primary cardiac neoplasms, and myxomas account for approximately 50% of all benign primary cardiac neoplasms.6 Lipomas are the second most common benign cardiac tumors and account for approximately 10% of all cardiac neoplasms.94,95 The most common intracardiac mass is a thrombus.23
- Secondary tumors are accepted to be more common than primary tumors by a factor of 20-30.
- Metastatic tumors to the heart and pericardium are more common than primary neoplasms and occur in as many as 1.5% of patients with malignancies.23 The most common sources of metastatic tumors are as follows (in approximate order of frequency): bronchogenic carcinomas of the breast; lymphomas; leukemia; the esophagus; the uterus; melanomas; the stomach; sarcomas; germ cell tumors; and tumors of the mouth and/or tongue, the colon and/or rectum, the kidneys, the thyroid, the larynx, the urinary bladder, the hepatobiliary system, the prostate, the pancreas, and the ovaries.96
International
The incidence of cardiac tumors internationally is identical to that in the United States.
Mortality/Morbidity
- Angiosarcomas have a high mortality rate. Most patients do not survive beyond 6 months from the time of diagnosis of this cardiac tumor.6,12
- Mortality rates in patients with other malignant cardiac tumors are also high, especially in those with metastases, which tend to involve the heart late in the course of malignant disease.
- Benign cardiac tumors can usually be resected and have a good prognosis.
Race
There is no known racial predisposition for cardiac tumors.
Sex
- Cardiac myxomas tend to occur more often in women than in men.6
- Cardiac lipomas have no sex predilection.23
- Rhabdomyosarcomas and angiosarcomas are more common in males than in females.6
Age
Patients with primary cardiac sarcomas have presented at age 1-75 years.38 Cardiac lipomas occur in patients of any age.23
- Pediatric tumors: Most primary pediatric tumors are diagnosed within the first year of life.83 Among pediatric patients, primary cardiac neoplasms include rhabdomyomas (78%), fibromas (11%), pericardial teratomas (2%), epicardial lipomas (2%), multicystic hamartomas (2%), and unspecified tumor types (5%).82 In children aged 15 years or younger, the most common benign primary cardiac tumors are rhabdomyomas (the most common benign primary tumor in patients <1 y of age, associated with tuberous sclerosis in 30-50% of patients), fibromas, hemangiomas, and teratomas. The most common malignant primary cardiac tumors in this age group are rhabdomyosarcoma fibrous histiocytoma, angiosarcoma, fibrosarcoma, and myxoid sarcoma.25,96
- Adult tumors: Angiosarcomas tend to be found in patients aged 20-50 years.70,97 About 70% of myxomas occur in middle-aged patients, usually those aged 50-70 years.6,23 In adults, the most common benign primary cardiac tumors are (in approximate order of frequency) myxomas (>50%), fibromas, hemangiomas, granular cell tumors, lipomas, paragangliomas, hamartomas, histiocytomas, and hemangioendotheliomas. The most common malignant primary cardiac tumors in this population are (in approximate order of frequency) angiosarcomas, fibrous histiocytomas, leiomyosarcomas, osteosarcomas, fibrosarcomas, myxoid sarcomas, rhabdomyosarcomas, and liposarcomas.12,96
Presentation
Patients with left heart disease may present with mitral valve obstruction or apparent subacute bacterial endocarditis, and those with right heart disease may present with tricuspid valve disease, pulmonary embolism, or hypertension.22
Atrial myxomas can cause breathlessness, fever, weight loss, syncope, hemoptysis, peripheral emboli, and sudden death.6
Angiosarcomas frequently involve the right heart and result in right-sided heart failure. Cardiac tamponade secondary to bloody pericardial effusions or tamponade with pleuritic chest pain may also be present.6,23,70
Intrapericardial masses can cause dyspnea as a result of airway compression.68,81
Patients with cardiac fibromas may present with congestive heart failure, arrhythmias, sudden death, cyanosis, or chest pain; one third of patients may be asymptomatic.98,99
Chest pain, palpitations, and flushing may be present in cases of intracardiac pheochromocytomas.21 Catecholamine release can cause irregular heartbeats, which can be detected by the patient as palpitations. Blood pressure can rise with an increased pulse rate, resulting in more work for the heart.
Lipomatous hypertrophy of the interatrial septum is frequently found in people with obesity (see Image 36).6
Most children with rhabdomyomas also have tuberous sclerosis, which is associated with adenoma sebaceum, seizures, and mental retardation.Preferred Examination
Initially, intracardiac tumors are best evaluated by using echocardiography. Differentiation of left atrial masses from thrombi may be best achieved by using transesophageal echocardiography. Because they provide a larger field of view, which affords a better opportunity to assess contiguous extracardiac involvement or the presence of metastatic disease, MRI, magnetic resonance angiography (MRA), and computed tomography (CT) are preferred over echocardiography for cases that are not as straightforward, or for cases in which acoustic access is restricted.100
Limitations of Techniques
Chest radiography may show the effects of intracardiac obstruction with features of pulmonary edema (see Images 1-2) but otherwise may contribute little. Echocardiography is easily accessible, but it is limited by restricted acoustic access. CT scanning is accurate but requires radiation and contrast material. In suitable patients, MRI has the fewest limitations, provided that the patient can remain motionless during the examination .
Differential Diagnoses
| Chondrosarcoma | Pheochromocytoma |
| Leiomyoma, Uterus (Fibroid) | Renal Cell Carcinoma |
| Liposarcoma, Soft Tissue | |
| Mesothelioma, Malignant | |
| Osteosarcoma, Variants |
Other Problems to Be Considered
Primary cardiac tumors
- Myxoma, fibroma, lipoma, rhabdomyoma, plasma cell granuloma, sarcoma, lymphoma, thymoma, hemangiopericytoma, fibroelastoma, mycosis fungoides, hamartoma/angiomyolipoma, hemangioma, angioma, lymphangioma, and pheochromocytoma
Primary cardiac sarcomas
- Angiosarcoma, osteosarcoma, fibrosarcoma, malignant fibrous histiocytoma, leiomyosarcoma, myxosarcoma, synovial sarcoma, neurofibrosarcoma, liposarcoma, lymphosarcoma, reticulum cell sarcoma, and undifferentiated sarcoma
Metastatic tumors
- Bronchogenic carcinoma, breast carcinoma, lymphoma, leukemia, carcinoid tumor, melanoma, cervical carcinoma, and germ cell tumors
Primary pediatric cardiac tumors
- Rhabdomyoma, intrapericardial teratoma, myxoma, fibroma, hemangioma, mesothelioma, multicystic hamartoma, epicardial lipoma, and rhabdomyosarcoma
Pericardial tumors
- Malignant spindle cell tumor, localized fibrous tumor (also termed localized fibrous mesothelioma), pericardial cyst, direct extension of primary cardiac tumors, or lung cancer
More on Cardiac Tumors |
 Overview: Cardiac Tumors |
| Imaging: Cardiac Tumors |
| Follow-up: Cardiac Tumors |
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| References |
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Further Reading
Keywords
cardiac tumor, heart tumor, heart neoplasm, primary cardiac tumors, secondary cardiac tumors, cardiac neoplasms, heart tumors, benign cardiac tumor, heart neoplasm, pericardial tumor, pericardial neoplasm, cardiac masses, pericardial masses, cardiac pseudotumors, myxoma, endodermal tumor, cardiac sarcoma, cardiac valve tumor, pediatric cardiac tumors, cardiac tumors in childhood, rhabdomyosarcoma, pediatric rhabdomyoma
