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Heart Tumors: Causes, Symptoms, Diagnosis, Treatment
Last reviewed: 18.10.2021
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Tumors of the heart can be primary (benign or malignant) or metastatic (malignant). Mixoma, a benign primary tumor, is the most frequent neoplasm of the heart. Tumors can develop from any tissue of the heart. They are capable of causing the obstruction of the valve or vesting tract, lead to thromboembolism, arrhythmia or pericardial pathology. Diagnosis is established by echocardiography and biopsy. Treatment of benign tumors usually involves surgical resection, often relapses. Treatment for metastatic lesions depends on the type of tumor and its origin, the prognosis is generally poor.
The incidence of primary heart tumors detected in autopsy is less than 1 per 2000 people. Metastatic tumors are found 30-40 times more often. Typically, primary heart tumors develop in the myocardium or endocardium, and they can also come from valvular tissues, connective tissue, or pericardium.
[Benign primary heart tumors
Benign primary tumors include myxomes, papillary fibroelastomas, rhabdomyomas, fibromas, hemangiomas, teratomas, lipomas, paragangliomas and pericardial cysts.
Mixoma is the most common tumor, accounting for 50% of all primary heart tumors. Prevalence in women is 2-4 times higher than in men, although in rare family forms (the Karnei complex), men suffer more often. Approximately 75% of the mix is localized in the left atrium, the rest - in other chambers of the heart as a single tumor or (more rarely) several formations. Approximately 75% are myxoma on the leg, they are able to prolapse through the mitral valve and impede ventricular filling during diastole. The other myxomes are single or spread on a wide base. Mixomes can be slime-like, smooth, hard and lobed or loose, and structureless. Friable unformulated myxomes increase the risk of systemic embolism.
The Karne complex is a hereditary, autosomal dominant syndrome characterized by the presence of recurrent cardiac mix, sometimes in combination with cutaneous myxomes, myxomatous fibroadenomas of the mammary glands, pigmented skin lesions (lentiginosis, freckles, blue nevi), multiple endocrine neoplasia (primary pigmented nodal adrenocortical disease, causing Cushing's syndrome, pituitary adenoma with excessive synthesis of growth hormone and prolactin, testicular tumors, adenoma of the thyroid gland or its cancer, cysts ovary) psammomatoznoy melanotic schwannoma, adenoma osteohondromiksomoy and mammary ducts. The age of diagnosing the disease is often young (an average of 20 years), patients have multiple myxomes (especially in the ventricles) and a high risk of recurrence of the myxoma.
Papillary fibroelastomy is the second most common benign primary tumor. These are avascular papillomas that predominate on the aortic and mitral valves. Men and women suffer equally often. Fibroelastomy has papillary branches that extend from the central nucleus, reminiscent of anemia. Approximately 45% - on the foot. They do not cause valvular dysfunction, but increase the risk of embolism.
Rabdomiomyas comprise 20% of all primary heart tumors and 90% of all cardiac tumors in children. Rhabdomyomas affect mainly infants and children, 50% of whom also have tuberous sclerosis. Rabdomiomy usually are multiple and located intramuralno in the septum or free wall of the left ventricle, where they damage the conduction system of the heart. The tumor is a hard white lobule, which usually regresses with age. A minority of patients develop tachyarrhythmias and heart failure due to obstruction of the outflow tract of the left ventricle.
Fibromas are also found mainly in children. They are associated with adenomas of the sebaceous glands of the skin and kidney tumors. Fibromas are more often present on the valve tissue and can develop in response to inflammation. They are able to squeeze or implant into the conduction system of the heart, causing arrhythmias and sudden death. Some fibromas are part of the syndrome with generalized body size increase, keratocytosis of the jaw, skeletal anomalies and various benign and malignant tumors (Gorlin's syndrome, or basal cell nevus).
Hemangiomas account for 5-10% of benign tumors. They cause symptoms in a small number of patients. Most often they are accidentally discovered during surveys conducted on a different occasion.
Teratomas of the pericardium are found mainly in infants and children. They are often attached to the base of large vessels. Approximately 90% are located in the anterior mediastinum, the rest mainly in the posterior mediastinum.
Lipomas can appear at any age. They are localized in the endocardium or epicardium and have a wide base. Many lipomas are asymptomatic, but some complicate blood flow or cause arrhythmias.
Paragangliomas, including pheochromocytomas, rarely occur in the heart; they are usually located at the base of the heart near the ends of the vagus nerve. These tumors may manifest as symptoms of catecholamine secretion.
Pericardial cysts may resemble a heart tumor or effusion into the pericardial cavity on the chest X-ray. Usually they are asymptomatic, although some cysts can cause compression symptoms (for example, chest pain, dyspnea, cough).
Malignant primary heart tumors
Malignant primary tumors include sarcomas, pericardial mesothelioma and primary lymphomas.
Sarcoma is the most frequent malignant and second in frequency primary heart tumor (after myxoma). Sarcomas develop mainly in middle-aged adults (mean age 41 years). Almost 40% of them are angiosarcomas, most of which develop in the right atrium and involve the pericardium, causing obstruction of the right ventricular tract, the pericardial tamponade and metastasis into the lungs. Other types include undifferentiated sarcoma (25%), malignant fibrous histiocyte (11-24%), leiomyosarcoma (8-9%), fibrosarcoma, rhabdomyosarcoma, liposarcoma and osteosarcoma. These tumors often appear in the left atrium, causing mitral valve obstruction and heart failure.
Mesothelioma of the pericardium is rare, can appear at any age, men are more likely than women. It causes a tamponade and can metastasize into the spine, adjacent soft tissues and the brain.
Primary lymphoma is extremely rare. Usually it develops in AIDS patients or other people with immunodeficiency. These tumors grow rapidly and cause heart failure, arrhythmias, tamponade and upper vena cava syndrome (SVPV).
Metastatic tumors of the heart
Carcinoma of the lungs and breast cancer, soft tissue sarcoma and kidney cancer are the most frequent sources of metastases in the heart. Malignant melanoma, leukemia and lymphoma are often metastasized in the heart, but metastases may not be clinically significant. When Kaposi's sarcoma spreads systemically in immunodeficient patients (usually with AIDS), it can spread to the heart, but clinically significant heart complications are rare.
Symptoms of heart tumors
Heart tumors cause symptoms typical of much more frequent diseases (eg, heart failure, stroke, IHD). Manifestations of benign primary heart tumors depend on the type of tumor, its location, size and the ability to decay. They are classified as non-cardiac, intramyocardial and intra-cavity.
Symptoms of non-cardiac tumors can be subjective or causing organic / functional changes. The first occur in the form of fever, chills, lethargy, arthralgia and weight loss are caused solely by myxomes, possibly as a result of the synthesis of cytokines (eg, interleukin-6); petechia is possible. These and other manifestations can be mistaken for the symptoms of infective endocarditis, connective tissue diseases and "mute" malignant growth. Another group of symptoms (eg, shortness of breath, chest discomfort) occurs due to compression of the heart cells or coronary arteries, irritation of the pericardium or cardiac tamponade caused by tumor growth or bleeding within the pericardium. Pericardial tumors can cause pericardial friction noise.
Symptoms of intramyocardial tumors include arrhythmias, usually atrio-ventricular or intraventricular blockage or paroxysmal supraventricular or ventricular tachycardias. The reason is tumors that are squeezed or implanted into the conducting system (especially rhabdomyomas and fibromas).
Manifestations of intracavitary tumors are caused by a violation of the functions of the valves and / or an obstruction of the blood flow (development of valvular stenosis, valvular insufficiency or heart failure), and in some cases (especially with myxomatous myxomes) - embolism, thrombi or tumor fragments in a large circle of circulation (brain, coronary arteries, kidneys, spleen, limbs) or lungs. Symptoms of intracavitary tumors can vary with a change in body position, which changes hemodynamics and physical forces that affect the tumor.
Mixams usually cause a certain combination of subjective and intracavitary symptoms. They can create a diastolic noise similar to that of mitral stenosis, but its loudness and localization change with each heartbeat and with a change in the position of the body. Approximately 15% of the foot mix localized in the left atrium produce a specific clapping sound as they slip into the mitral orifice during diastole. Miksoms can also cause arrhythmias. The phenomenon of Raynaud and the thickening of the terminal phalanges of the fingers are less characteristic, but possible.
Fibroelastomas, often detected accidentally during autopsy, are usually asymptomatic, but they can be a source of systemic embolism. Rhabdomyomas are often not accompanied by clinical symptoms. Fibromas lead to arrhythmias and sudden death. Hemangiomas are usually asymptomatic, but can cause any of the non-cardiac, intramyocardial or intracavitary symptoms. In teratomas, there is a respiratory distress syndrome and cyanosis due to compression of the aorta and pulmonary artery or WPW syndrome.
Manifestations of malignant cardiac tumors occur more sharply and rapidly progress. Cardiac sarcomas usually cause symptoms of obstruction of the vestibular tract and cardiac tamponade. Mesothelioma leads to the appearance of signs of pericarditis or cardiac tamponade. Primary lymphoma causes refractory progressive heart failure, tamponade, arrhythmias and WPW syndrome. Metastatic cardiac tumors can manifest as a sudden enlargement of the heart, its tamponade (due to rapid accumulation of hemorrhagic effusion in the pericardial cavity), heart block, other arrhythmias or sudden unexplained heart failure. Also possible fever, malaise, weight loss, night sweats and loss of appetite.
Where does it hurt?
Diagnosis of heart tumors
The diagnosis, often delayed, because the clinical manifestations are similar to the symptoms of much more frequent diseases, are confirmed by echocardiography and tissue typing in biopsy. Transesophageal echocardiography better visualizes atrial tumors, and transthoracic - ventricular. If the results are doubtful, radioisotope scanning, CT or MRI is used. Sometimes contrast ventriculography is necessary during cardiac catheterization. A biopsy is performed during catheterization or open thoracotomy.
With myxomes, extensive examinations often precede echocardiography, since the symptoms of the disease are nonspecific. Often there are anemia, thrombocytopenia, leukocytosis, increased ESR, content of C-reactive protein and y-globulin. ECG data can indicate an increase in the left atrium. Routine chest X-ray in some cases demonstrates calcium deposits in myxomes of the right atrium or teratomas, revealed in the form of formations in the anterior mediastinum. Sometimes myxomes are diagnosed when tumor cells are detected in surgically removed emboli.
Arrhythmias and heart failure with features of tuberous sclerosis testify to rhabdomyomas or fibromas. New cardiac symptoms in a patient with a diagnosed, non-cardiac malignancy suggest metastases in the heart; Chest X-ray can show bizarre changes in the contours of the heart.
What do need to examine?
Treatment of heart tumors
Treatment of benign primary tumors - surgical excision with subsequent periodic echocardiography at least 5-6 years for the timely diagnosis of relapse. Tumors are excised if another disease (eg, dementia) does not become a contraindication to surgical intervention. The operation usually gives good results (95% survival for 3 years). Exceptions are rhabdomyomas, most of which regress spontaneously and do not require treatment, and pericardial teratomas that can lead to the need for urgent pericardiocentesis. Patients with fibroelastoma may need a valve or prosthetic repair. When rhabdomyomas or fibroids are multiple, surgical treatment is usually ineffective, and the prognosis is poor within 1 year after 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. It may include systemic chemotherapy or palliative procedures.