Key points
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Primary cardiac tumors (PCTs) are extremely rare that may be symptomatic or found incidentally.
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The signs and symptoms of cardiac tumors generally are determined by the location of the tumor in the heart and not by its histopathology.
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Cardiac myxoma is the most common benign heart tumor.
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The tumor plop sound is one of the classic and characteristic auscultation findings of cardiac myxoma. The other physical examination findings in cardiac tumors include prominent A wave with elevation of JVP; loud S1, S3, S4; and diastolic rumble.
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Malignant tumors are extremely rare and represent only 5% to 6% of PCTs. The most common are sarcomas.
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Cardiac metastases are 20–40 times more common than PCTs.
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Melanomas have the greatest propensity for cardiac involvement, and also carcinomas of the thorax, including breast, lung, and esophageal, are the most common carcinomas that metastasize to the heart.
Primary cardiac tumors (PCTs) are very rare . As an example, in one series of over 12,000 autopsies, only seven were identified with an incidence of less than 0.1% . Cardiac tumors may be symptomatic or found incidentally. In symptomatic patients, a mass can virtually always be detected by echocardiography, magnetic resonance imaging, and/or computed tomography. Because symptoms may mimic other cardiac conditions, the clinical challenge is to consider the possibility of a cardiac tumor so that the appropriate diagnostic test(s) can be conducted.
Clinical manifestations
The signs and symptoms of cardiac tumors generally are determined by the location of the tumor in the heart and not by its histopathology .
Mechanisms of symptom production
Cardiac tumors may cause symptoms through different mechanisms:
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Embolization which is usually systemic but can be pulmonic. Aortic valve and left atrial tumors were associated with greatest risk of embolization .
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Obstruction of the circulation through the heart or heart valves, producing symptoms of heart failure. Interference with the heart valves, causing regurgitation.
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Direct invasion of the myocardium, resulting in impaired left ventricular function, arrhythmias, heart block, or pericardial effusion with or without tamponade.
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Invasion of the adjacent lung may cause pulmonary symptoms and may mimic bronchogenic carcinoma. Constitutional or systemic symptoms.
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Left atrial tumors may release tumor fragments or thrombi into the systemic circulation and lead to neurological complications.
Physical examination of cardiac tumors
| Findings | Comments | |
|---|---|---|
| Neck | Prominent A wave with elevation of JVP | |
| Heart | Loud S1 | Prolapsing of atrial tumor into the mitral valve orifice results in delay in closure of mitral valve producing |
| Delay in P2 | Intensity of which depends on the absence or presence of pulmonary hypertension | |
| Tumor plop | Trial tumor striking against the endocardial wall may produce an early diastolic sound | |
| S3 and S4 | In some cases S3 and S4 may also be present | |
| Murmur | Diastolic atrial rumble | Obstruction of mitral valve by the atrial tumor |
| Systolic murmur at cardiac apex | Damaging of the mitral valves leading to mitral regurgitation | |
| Diastolic rumble | Obstruction of the tricuspid valve and a holosystolic murmur due to tricuspid regurgitation in right atrial tumors |
Histology of benign cardiac tumors
| Tumors | Histopathology | References |
|---|---|---|
| Myxoma | Spindle or stellate cells, pseudovascular structure, myxoid matrix, hemorrhage, dystrophic calcification can be present | |
| Lipoma | Mature adipocytes, occasionally with entrapped myocytes at the periphery | |
| Fibroma | Fibroblasts and collagen bundles, some elastic fibers, calcification is a common finding | |
| Rhabdomyoma | Spider cell (vacuolated enlarged cardiac myocyte with clear cytoplasm due to abundant glycogen) | |
| Papillary fibroelastoma | Endocardium-coated fronds with an avascular collagenous core containing mucopolysaccharide and elastin |
Most PCTs are benign and include myxomas, rhabdomyomas, papillary fibroelastomas, fibromas, hemangiomas, lipomas, and leiomyomas. Myxoma is the most common pathological subtype . Nonmyxoma subtypes, which mostly occur in children and adolescents, are less reported . Benign tumors have favorable prognosis with a 30-day mortality of only 1% . They are generally more common in older women , and according to their size and location, benign PCTs manifest with a wide array of symptoms. However, 13.3%–27.7% of cases occur in asymptomatic individuals and are detected incidentally .
Cardiac myxomas are the most common PCT and be derived from mesenchymal cell precursors . They form intracavitary masses, which are most commonly found in the left atrium attached by a stalk to the fossa ovalis and also may be seen in the right atrium in children . Myxomas are morphologically divided into two groups: polypoid and papillary. The former, when large, may present with obstructive symptoms with a “tumor plop” being occasionally heard on auscultation. In contrast, papillary myxoma causes embolic events. In both variants, constitutional symptoms like fatigue, fever, and weight loss have also been reported. Calcification is seen in approximately 14% of patients and is more commonly associated in right-sided lesions .
| Symptoms | Incidence (%) |
|---|---|
| Dvsonea on exertion | > 75 |
| Paroxysmal dyspnea | − 25 |
| Fever | − 50 |
| Weight loss | − 25 |
| Severe dizziness/syncope | − 20 |
| Sudden death | − 15 |
| Hemoptysis | − 15 |
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