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Heart tumors: causes, symptoms, diagnosis, treatment
Last reviewed: 07.07.2025
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Cardiac tumors may be primary (benign or malignant) or metastatic (malignant). Myxoma, a benign primary tumor, is the most common neoplasm of the heart. Tumors can develop from any tissue in the heart. They can cause valve or outflow tract obstruction, thromboembolism, arrhythmia, or pericardial pathology. Diagnosis is by echocardiography and biopsy. Treatment of benign tumors usually involves surgical resection; relapses are common. Treatment of metastatic lesions depends on the type of tumor and its origin; the prognosis is generally poor.
The incidence of primary cardiac tumors detected at autopsy is less than 1 in 2,000 people. Metastatic tumors are found 30 to 40 times more often. Primary cardiac tumors usually develop in the myocardium or endocardium, but they can also originate from valve tissue, connective tissue, or the pericardium.
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Benign primary cardiac tumors
Benign primary tumors include myxomas, papillary fibroelastomas, rhabdomyomas, fibromas, hemangiomas, teratomas, lipomas, paragangliomas, and pericardial cysts.
Myxoma is the most common tumor, accounting for 50% of all primary cardiac tumors. The prevalence in women is 2-4 times higher than in men, although in rare familial forms (Carney complex), men are affected more often. About 75% of myxomas are localized in the left atrium, the rest - in other chambers of the heart as a single tumor or (less often) several formations. About 75% of myxomas are pedunculated; they can prolapse through the mitral valve and impede ventricular filling during diastole. The remaining myxomas are solitary or spreading on a broad base. Myxomas may be mucoid, smooth, hard and lobulated, or friable and structureless. Friable, unformed myxomas increase the risk of systemic embolism.
Carne complex is a hereditary, autosomal dominant syndrome characterized by the presence of recurrent cardiac myxomas, sometimes in combination with cutaneous myxomas, myxomatous fibroadenomas of the mammary glands, pigmented skin lesions (lentigines, freckles, blue nevi), multiple endocrine neoplasia (primary pigmented nodular adrenocortical disease causing Cushing's syndrome, pituitary adenoma with excessive synthesis of growth hormone and prolactin, testicular tumors, thyroid adenoma or its cancer, ovarian cysts), psammomatous melanotic schwannoma, mammary duct adenoma and osteochondromyxoma. The age at diagnosis of the disease is often young (on average 20 years), patients have multiple myxomas (especially in the ventricles) and a high risk of myxoma recurrence.
Papillary fibroelastomas are the second most common benign primary tumor. They are avascular papillomas that predominate on the aortic and mitral valves. Men and women are affected equally. Fibroelastomas have papillary branches extending from a central core, resembling sea anemones. Approximately 45% are pedunculated. They do not cause valvular dysfunction, but increase the risk of embolism.
Rhabdomyomas account for 20% of all primary cardiac tumors and 90% of all cardiac tumors in children. Rhabdomyomas affect mainly infants and children, 50% of whom also have tuberous sclerosis. Rhabdomyomas are usually multiple and located intramurally in the septum or free wall of the left ventricle, where they damage the cardiac conduction system. The tumor appears as firm, white lobules that typically regress with age. A minority of patients develop tachyarrhythmias and heart failure due to left ventricular outflow tract obstruction.
Fibromas also occur mainly in children. They are associated with sebaceous gland adenomas of the skin and renal tumors. Fibromas are more often present on valve tissue and may develop in response to inflammation. They can compress or invade the cardiac conduction system, causing arrhythmia and sudden death. Some fibromas are part of a syndrome with generalized increase in body size, keratocytosis of the jaw, skeletal anomalies, and various benign and malignant tumors (Gorlin syndrome, or basal cell nevus).
Hemangiomas account for 5-10% of benign tumors. They cause symptoms in a small number of patients. They are most often discovered incidentally during examinations performed for another reason.
Pericardial teratomas occur primarily in infants and children. They are often attached to the base of large vessels. Approximately 90% are located in the anterior mediastinum, the remainder mainly in the posterior mediastinum.
Lipomas can appear at any age. They are localized in the endocardium or epicardium and have a broad base. Many lipomas are asymptomatic, but some obstruct blood flow or cause arrhythmia.
Paragangliomas, including pheochromocytomas, are rare in the heart; they are usually located at the base of the heart near the endings of the vagus nerve. These tumors may present with symptoms of catecholamine secretion.
Pericardial cysts may resemble a cardiac tumor or pericardial effusion on chest radiograph. They are usually asymptomatic, although some cysts may cause compressive symptoms (eg, chest pain, shortness of breath, cough).
Malignant primary cardiac tumors
Malignant primary tumors include sarcomas, pericardial mesothelioma, and primary lymphomas.
Sarcoma is the most common malignant and the second most common primary cardiac tumor (after myxoma). Sarcomas occur primarily in middle-aged adults (mean age 41 years). Almost 40% are angiosarcomas, most of which arise in the right atrium and involve the pericardium, causing right ventricular inflow tract obstruction, pericardial tamponade, and pulmonary metastasis. Other types include undifferentiated sarcoma (25%), malignant fibrous histiocytoma (11–24%), leiomyosarcoma (8–9%), fibrosarcoma, rhabdomyosarcoma, liposarcoma, and osteosarcoma. These tumors most commonly arise in the left atrium, causing mitral valve obstruction and heart failure.
Pericardial mesothelioma is rare, can occur at any age, and affects men more often than women. It causes tamponade and can metastasize to the spine, adjacent soft tissues, and the brain.
Primary lymphoma is extremely rare. It usually develops in people with AIDS or other immunocompromised individuals. These tumors grow rapidly and cause heart failure, arrhythmias, tamponade, and superior vena cava syndrome (SVCS).
Metastatic cardiac tumors
Lung and breast carcinoma, soft tissue sarcoma, and renal cell carcinoma are the most common sources of cardiac metastasis. Malignant melanoma, leukemia, and lymphoma frequently metastasize to the heart, but the metastases may not be clinically significant. When Kaposi sarcoma spreads systemically in immunocompromised patients (usually those with AIDS), it may spread to the heart, but clinically significant cardiac complications are rare.
Symptoms of Heart Tumors
Cardiac tumors cause symptoms typical of much more common diseases (e.g., heart failure, stroke, coronary heart disease). The manifestations of benign primary cardiac tumors depend on the type of tumor, its location, size, and ability to disintegrate. They are classified as extracardiac, intramyocardial, and intracavitary.
Symptoms of extracardiac tumors may be subjective or organic/functional. The former, fever, chills, lethargy, arthralgias, and weight loss, are caused exclusively by myxomas, possibly by cytokine synthesis (eg, interleukin-6); petechiae may be present. These and other manifestations may be mistaken for infective endocarditis, connective tissue disease, and silent malignancy. Other symptoms (eg, dyspnea, chest discomfort) result from compression of cardiac chambers or coronary arteries, pericardial irritation, or cardiac tamponade caused by tumor growth or hemorrhage within the pericardium. Pericardial tumors may cause pericardial friction rubs.
Symptoms of intramyocardial tumors include arrhythmias, usually atrioventricular or intraventricular block or paroxysmal supraventricular or ventricular tachycardias. The cause is tumors that compress or invade the conduction system (especially rhabdomyomas and fibromas).
Manifestations of intracavitary tumors are caused by dysfunction of the valves and/or obstruction of blood flow (development of valve stenosis, valve insufficiency or heart failure), and in some cases (especially with myxomatous myxomas) - embolism, thrombi or tumor fragments in the systemic circulation (brain, coronary arteries, kidneys, spleen, limbs) or lungs. Symptoms of intracavitary tumors may vary with a change in body position, which changes the hemodynamics and physical forces affecting the tumor.
Myxomas usually cause some combination of subjective and intracavitary symptoms. They may produce a diastolic murmur similar to that of mitral stenosis, but its loudness and location vary with each heartbeat and with body position. About 15% of pedunculated myxomas located in the left atrium produce a characteristic popping sound as they slip into the mitral orifice during diastole. Myxomas may also cause arrhythmias. Raynaud's phenomenon and clubbing of the fingers are less common but possible.
Fibroelastomas, often discovered incidentally at autopsy, are usually asymptomatic but may be a source of systemic embolism. Rhabdomyomas are most often asymptomatic. Fibromas cause arrhythmias and sudden death. Hemangiomas are usually asymptomatic but may cause any of the extracardiac, intramyocardial, or intracavitary symptoms. Teratomas cause respiratory distress and cyanosis due to compression of the aorta and pulmonary artery or WPW syndrome.
The manifestations of malignant cardiac tumors are more acute and progressive. Cardiac sarcomas typically cause symptoms of ventricular inflow tract obstruction and cardiac tamponade. Mesothelioma causes features of pericarditis or cardiac tamponade. Primary lymphoma causes refractory progressive heart failure, tamponade, arrhythmias, and WPW syndrome. Metastatic cardiac tumors may present with sudden cardiac enlargement, tamponade (due to rapid accumulation of hemorrhagic effusion in the pericardial cavity), heart block, other arrhythmias, or sudden unexplained heart failure. Fever, malaise, weight loss, night sweats, and loss of appetite may also occur.
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Diagnosis of cardiac tumors
The diagnosis, often delayed because the clinical manifestations resemble those of much more common diseases, is confirmed by echocardiography and tissue typing during biopsy. Transesophageal echocardiography better visualizes atrial tumors, and transthoracic echocardiography better visualizes ventricular tumors. If the results are questionable, radioisotope scanning, CT, or MRI are used. Contrast ventriculography during cardiac catheterization is sometimes necessary. Biopsy is performed during catheterization or open thoracotomy.
In myxomas, extensive examinations often precede echocardiography, since the symptoms of the disease are nonspecific. Anemia, thrombocytopenia, leukocytosis, increased ESR, C-reactive protein, and γ-globulins are often present. ECG data may indicate left atrial enlargement. Routine chest radiography in some cases demonstrates calcium deposits in myxomas of the right atrium or teratomas detected as masses in the anterior mediastinum. Myxomas are sometimes diagnosed when tumor cells are found in surgically removed emboli.
Arrhythmias and heart failure with features of tuberous sclerosis suggest rhabdomyomas or fibromas. New cardiac symptoms in a patient with a known extracardiac malignancy suggest cardiac metastases; chest radiography may show bizarre changes in cardiac contour.
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Treatment of cardiac tumors
Treatment of benign primary tumors is surgical excision followed by periodic echocardiography for at least 5-6 years for timely diagnosis of recurrence. Tumors are excised unless another disease (eg, dementia) contraindicates surgery. Surgery usually gives good results (95% survival at 3 years). Exceptions include rhabdomyomas, most of which regress spontaneously and do not require treatment, and pericardial teratomas, which may require urgent pericardiocentesis. Patients with fibroelastoma may require valve repair or replacement. When rhabdomyomas or fibromas are multiple, surgical treatment is usually ineffective and the prognosis is poor within 1 year of diagnosis; 5-year survival may be below 15%.
Treatment of malignant primary tumors is usually palliative (eg, radiation therapy, chemotherapy, treatment of complications) because the prognosis is poor.
Therapy for metastatic cardiac tumors depends on the origin of the tumor and may include systemic chemotherapy or palliative procedures.