AUTHOR AND EDITOR INFORMATION

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• Workup
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• Follow-up
• Miscellaneous
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INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
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Background

Heart tumors were first described in the 18th century by Boneti; however, many believe the description by Albers in 1835 is the first authentic report. In 1936, the first successful removal of a neoplasm of the heart was performed. In 1952, angiography was first used for the diagnosis of heart tumors, and, in 1955, bypass was used for the first time to excise an intracavitary tumor. Cardiac tumors, whether primary or metastatic, are rare.

Benign neoplasms occur 3 times more often than malignant tumors. By far, the most common variety of tumors reported in children and adolescents is rhabdomyoma, followed by fibroma, myxoma, and teratoma. Sarcoma is the largest group of primary cardiac malignant neoplasms. Of these sarcomas, angiosarcomas are the most common histologic type and occur more frequently in males. These tumors seed blood directly, thus metastases are common and widespread. Signs and symptoms of these tumors at presentation are generalized, nonspecific, and mimic several other systemic diseases.

The clinical presentation of a patient with a cardiac tumor is determined more by the tumor's location than by its histologic type. Benign tumors, depending on their location, can present more symptoms than malignant tumors if they critically obstruct a valve or outflow tract. It is more important to consider the findings typical for each location.

Right-sided tumors may present with congestive heart failure (CHF) manifested by fatigue, edema, jugular venous distention, and ascites. Other symptoms include shortness of breath, syncope, and night sweats. Pericardial effusions may occur. Vena cava syndrome, pulmonary embolism, and restrictive cardiomyopathy are some of the complications.

Left atrial and left ventricular tumors can present a variety of signs and symptoms that include, but are not restricted to, fever, chills, dizziness, dyspnea on exertion, cold sweats during exercise or at night, and nonproductive cough. Since tumors may embolize, they also can lead to seizures, transient ischemic attacks, and cerebrovascular and peripheral-vascular accidents. Based on their size and position, they may induce arrhythmias and interfere with ventricular compliance.

Benign tumors

Rhabdomyomas are hamartomas and are the most frequently found tumors in children. They are associated with tuberous sclerosis in about 50-80% of patients. Of patients with tuberous sclerosis, 50-60% have rhabdomyomas. These tumors are frequently multiple, involving ventricular free and septal walls, and have a yellowish-gray color. They vary from small to extremely large. They rarely are excised because they tend to regress over time; if patients are asymptomatic, only observation is warranted. Surgical removal is indicated when they present with obstruction leading to cardiac compromise or intractable arrhythmias.

Fibromas, usually single and large, most commonly are found in the left ventricular free wall and rarely involve the septal wall. As many as 40% of fibromas are diagnosed in infants younger than 1 year. These tend to be firm nonencapsulated tumors derived from fibroblasts.

Myxomas are usually seen in adults. They rarely are seen in children, accounting for only 9-15% of all cardiac tumors from birth to adolescence. They often are found attached to the atrial septum and mitral valve apparatus in the left atrium (>85%). Myxomas can appear sporadically or as part of the syndrome myxoma or Carney syndrome, which includes endocrine neoplasms, tumors in other organs, and skin with spotty hyperpigmentation. This type of familial cardiac myxoma accounts for less than 10% of the myxomas appearing in the heart. They have an autosomal dominant transmission and most commonly appear in females. Patients with syndrome myxoma tend to be younger than those with sporadic myxomas. Myxomas may embolize; this may be their first clinical presentation. Peripheral embolization is reported to occur in as many as 70% of patients with myxomas and may even occur in utero.

Teratomas are single, encapsulated, grayish-tan tumors that appear most often in the pericardium. Teratomas develop in the right atrium, right ventricle, and septum of the heart. They are large basal tumors that, in newborns, may be larger than the patient's own heart.

Angiomas are a benign proliferation of endothelial cells, also known as vascular tumors. They can occur in any part of the heart (with a preference for right-sided chambers) and can form blood vessels (hemangiomas) or lymph vessels (lymphangiomas). These vascular vessels communicate between themselves within the myocardium. They can infiltrate the intraventricular septum near the conduction system where they may cause heart block. Hemangiomas are red, hemorrhagic, sessile, or polypoid subendocardial nodules that vary from small to large and occasionally have been associated with hemorrhagic cardiac tamponade.

Other types of tumors that have been less frequently reported in children include lipomas, papillary tumors, accessory cardiac cushion tissue, leiomyomas, mesotheliomas, fibroelastomas, fibroelastic papillomas, and benign cystic tumors.

Malignant tumors

Sarcomas originate from mesenchyme and, therefore, display a wide variety of morphologic types. These tumors are rare in children, with angiosarcoma being the most common type of sarcoma for all ages.

Cardiac angiosarcomas characteristically are lobulated variegated masses that are necrotic or hemorrhagic and are composed of anastomosing vascular channels lined by malignant cells that may contain areas of spindle cells. They tend to be aggressive malignancies, either infiltrative or polypoid, with most arising from the right atrium. Metastases are common and, based on autopsy studies, occur in as many as 88% of patients with malignant tumors.

Rhabdomyosarcomas grow invasively, metastasize, and can recur. These tumors are rare and are more common in children because they may arise from embryonic cell rests in the septum.

Fibrosarcomas, often involving more than one chamber of the heart, contain areas of hemorrhage and necrosis.

Other malignant neoplasms

Other reported malignant cardiac tumors are lymphomas, histiocytoma, leiomyosarcomas, choriocarcinoma, liposarcoma, and osteogenic sarcomas.

Frequency

United States

Primary cardiac tumors are rare, with a frequency of 0.0017-0.28% based on autopsies. About 75% of them are benign and 25% are malignant. Secondary (metastatic) tumors are 10-40 times more frequent. Lam et al reviewed 12,485 autopsies and found only 7 cases of primary neoplasm of the heart versus 154 cases of secondary heart tumors.

Rhabdomyomas are the most common tumor in children, with approximately 75% diagnosed in children younger than 1 year. Of these tumors, 50-80% are associated with tuberous sclerosis.

Fibromas are the second most common tumor in children, with most (40%) diagnosed in children younger than 1 year.

Myxomas are seen in 9-15% of patients with cardiac tumors, are the most common primary cardiac neoplasms in all ages, and comprise 50-60% of all heart tumors.

The true incidence of teratomas is not known; however, 50% are diagnosed in newborns and 66% in children younger than 1 year.

Sarcomas comprise 25% of all cardiac tumors; the most frequent tumor found is the angiosarcoma.

Race

No racial predilection exists.

Sex

Myxomas are more common in females, especially in patients with syndrome myxoma or Carney syndrome. Angiosarcomas have a 2:1 male-to-female ratio. Rhabdomyomas, fibromas, teratomas, other sarcomas, and malignant tumors do not have any sex predilection.

Age

No specific age predilection exists; however, rhabdomyomas, fibromas, teratomas, and rhabdomyosarcomas are more commonly seen in children and adolescents than in adults. Myxomas comprise 50-60% of all benign tumors of the heart and are found mainly in adults.

CLINICAL

Section 3 of 11  

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History

Diagnosis is a challenge for any physician because cardiac masses have no typical presentation. Typically, patients are asymptomatic or present with nonspecific signs and symptoms. Some authors call heart neoplasms the great masqueraders. Certain symptoms, including irritability, shortness of breath, anorexia, tiredness, or palpitations, may raise suspicion of a neoplastic process.

• Initial symptoms in infants may include irritability, periodic episodes of pallor, fever, tachypnea, tachycardia, anorexia, and failure to thrive.
• Older children and adolescents may present with similar symptoms and may complain of dyspnea on exertion, dizziness, general malaise, syncope, hemoptysis, and shock or experience sudden death.
• For presenting symptoms of specific types of cardiac tumors, see Background.

Physical

Clinical presentation and physical findings relate to location of the tumor. Arrhythmias, heart failure, fever, pericardial effusion, and new or louder heart murmurs are a few of the findings for all these growths (see Background).

• Right-sided tumors: Heart failure, edema, jugular venous distention, ascites, shortness of breath, right-sided third and/or fourth heart sounds, cor pulmonale, pericardial effusions, hepatomegaly, vena cava syndrome, and pulmonary embolism are associated with right-sided tumors.
• Left-sided tumors: With the ability to embolize, these tumors can lead to seizures, transient ischemic attacks, and cerebrovascular and peripheral-vascular accidents.
• Based on their size and position, left- and right-sided tumors may interfere with ventricular compliance.
• Nonproductive cough and hemoptysis have been reported in older children and adults.
• Malar flush, emboli, spotty hyperpigmentation of the skin, and a distinctive apical diastolic sound called a tumor plop are associated with myxoma.

Causes

Recently, mutations in the gene protein kinase A (PKA) were identified in patients with Carney complex and myxomas. A mutation of the gene that causes neurofibromatosis (NF) is present in patients who have NF and cardiac tumors. Whether or not this means cardiac tumors are directly related to NF is uncertain, though the chances of that being the case are good.

DIFFERENTIALS

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Other Problems to be Considered

Collagen vascular diseases
Mural thrombus
Cerebrovascular accident
Vasculitis (ie, lupus, polyarteritis)
Mitral valve stenosis

WORKUP

Section 5 of 11  

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• Workup
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• Follow-up
• Miscellaneous
• Multimedia
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Lab Studies

• Complete blood count (CBC) with differential should be normal in benign heart tumors. In malignant tumors, any kind of blood count abnormality can be present in these patients. For example, anemia is not an uncommon finding. Platelet count may be elevated or low, and WBC count may be high.
• Erythrocyte sedimentation rate (ESR) is a nonspecific marker for inflammation that may be normal or elevated; however, the ESR could be low in the presence of CHF.
• Liver function test findings occasionally are elevated because of severe right-sided heart failure or metastases.
• Creatine kinase-myocardial band (CK-MB) fraction levels have been found to be higher in patients who have both ventricular dysfunction and a cardiac tumor, but not necessarily primary malignancies from the heart.

Imaging Studies

• Echocardiogram
• • This is the most cost-effective of all tests used to evaluate the anatomy of the heart. An echocardiogram can provide important information about size, extension and/or invasion within and outside the heart, valvar involvement and/or competency, ventricular function, and pericardial effusion.
  • It is very sensitive but not specific.
  • Some of these tumors may be too small for echocardiographic detection, especially those leading to dysrhythmias.
  • Transesophageal echocardiogram also is useful in detecting tumors in the left heart, especially in adults.
• Chest roentgenogram: Cardiomegaly and pulmonary edema may present in cases of obstructive lesions. Certain neoplasms can have calcium deposition detected on the roentgenogram.
• Magnetic resonance imaging (MRI) is a powerful tool in diagnosing tumors, evaluating their extent, and seeking metastases. It may delineate the area of the mass better than echocardiography because it has a larger field of observation. Contrast enhancement and multislice imaging can provide a three-dimensional view of tissue. It also can help differentiate tumors from thrombi.
• Computed tomography (CT) scan: Ultrafast CT scan is gaining use in this area since it eliminates artifacts of heart motion seen with conventional CT scanning. CT is a powerful tool in evaluating the extent of the tumor and exhibits a high degree of tissue discrimination.

Other Tests

• Electrocardiogram
• • Nonspecific changes are the most common findings; however, ST-T segment changes with strain patterns have been noticed in obstructive rhabdomyomas and fibromas. After surgery, these changes return to normal. Other patterns associated with ischemia also may be present.
  • Diffuse low-voltage QRS, especially in the presence of pericardial effusion, can be seen.
  • Arrhythmias may be the first presentation of an intracardiac tumor and occasionally are resistant to medical therapy.
  • Other findings include intraventricular conduction delay and complete bundle branch block.

Procedures

• Endomyocardial biopsy is controversial and seldom useful in children because of the high possibility of false-negative results and the high possibility of metastasis during the procedure if a malignant tumor is present.

Histologic Findings

Benign tumors

Rhabdomyomas are a glycogenic degeneration of myocardial fibers and thus are considered hamartomas. They contain large vacuolated cells filled with glycogen. Their microscopic hallmark, the spider cell, displays eccentric nuclei, granular cytoplasm with a central cytoplasmic mass, and fine fibrillar processes that radiate to the periphery, giving the appearance of a spider in a web. Since the cytoplasm is rich in glycogen, it stains positive with periodic acid-Schiff reagent. Rhabdomyositis is a rare form of cardiomyopathy. In this pathology, microscopic changes in the muscle and conduction system are evident, yet gross anatomic findings are rare.

Cardiac fibromas consist of elongated fibroblasts, with fibrous tissue and collagen. Occasionally, calcium can be seen in radiographs or microscopically within the tumor. Mitosis rarely is seen in this type of tumor.

Myxoma has a characteristic pattern of cells termed lipidic cells embedded in a myxoid stroma rich in glycosaminoglycans. These cells are multinucleated with pink cytoplasm. Typically, they present in clusters surrounding vascular structures. Calcium is present in approximately 10% of patients. Usually, the embolized material has a different pattern from the material at the center of the lesion, and cells resemble embryonic mesenchymal cells, similar to embryonic endocardial cushion tissue.

Teratomas, rarely malignant in children, form from 3 embryonic tissues that usually grow in the anterior mediastinum. These tumors should be differentiated from intrapericardial bronchogenic cysts because they may have the same gross appearance; the histologic difference is that bronchogenic cysts do not contain neuronal tissue, which is usually present in teratomas.

Hemangiomas are classified, based on the predominant type of proliferating vascularity, into hemangiomas (common) or lymphangiomas (extremely uncommon). The tumor contains endothelium-lined spaces that may have blood, lymph, or thrombi.

Malignant tumors

Angiosarcomas have variable vascular channels lined with atypical endothelial cells.

The histologic hallmark of rhabdomyosarcoma is the presence of cross-striations.

Fibrosarcomas are composed of a herringbone pattern of spindle-shaped cells with elongated blunt-ended nuclei and frequent mitoses.

TREATMENT

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Medical Care

Most childhood cardiac neoplasias are benign with no treatment necessary unless the tumor severely obstructs blood flow or causes intractable arrhythmias. Most only require close follow-up care.

New data have addressed the use of serum CK-MB fraction levels in evaluating ventricular function. Greater ventricular dysfunction correlated with higher CK-MB levels.

Nonetheless, examine the patient extensively and expeditiously since finding a tumor is a stressful situation for both patient and family. By reaching a prompt and precise diagnosis, the physician can proceed with the best treatment approach.

The mode of treatment is variable and cannot be simplified easily since the kind of tumor (benign vs malignant, infiltrative vs localized) dictates therapy. Location and extent of the tumor, as well as symptoms, are clinical variables that direct treatment. Observation is sufficient when the mass is small and does not interfere with vascular hemodynamics. The clinician must be aggressive, however, when the tumor is causing hemodynamic problems.

Surgical Care

Many advocate excision of the mass as soon as it is found; however, most childhood tumors are benign and do not require resection. Nevertheless, if a tumor is causing severe obstruction or intractable arrhythmias and has been rendered resectable, perform surgery as soon as possible.

• Excision and biopsy: If excision is required, perform it as extensively and completely as possible. Certain tumors recur even after complete excision. An extensive resection carries its own risks since, occasionally, removal of papillary muscles, valves, chordae tendineae, and conduction tissue are necessary.
• Heart transplantation is an option for unresectable benign masses causing hemodynamic compromise. Transplantations have been performed with variable results for primary malignant cardiac tumors in children and adults without evidence of metastases.

Consultations

Consult a cardiologist, thoracic and cardiovascular surgeon, pathologist, radiologist, and hematologist/oncologist.

Diet

No dietary restrictions are needed. Occasionally, certain restrictions such as low-sodium diet for CHF are required.

Activity

Advise no restrictions unless clinical condition merits otherwise.

MEDICATION

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Focus medical treatment of a patient with a cardiac neoplasm on symptoms and possible complications resulting from primary disease. Two of the more common complications of patients with heart tumors are development of arrhythmias and CHF.

CHF management includes diuretics and afterload reduction. Severe cases with hemodynamic compromise may require intravenous inotropes, afterload reduction, vasodilators, and anticoagulation. Arrhythmias are variable; thus, specific antiarrhythmics should be chosen.

Drug Category: Cardiac glycosides

These agents may improve left ventricular function by inhibiting the Na⁺/K⁺-ATPase activity that results in increased myocardial contraction. This leads to sodium accumulation within the myocyte that stimulates the sodium calcium exchange pump. Increased intracellular calcium increases the force of contraction while producing antiarrhythmic effects on cardiac muscle.

Drug Category: Diuretic agents

Hypoperfusion of kidneys causes retention of sodium and water, producing peripheral and pulmonary edema. Diuretics decrease intravascular volume overload. They promote excretion of water and electrolytes by the kidneys and are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention has resulted in edema or ascites. Pay special attention to patients with a combination of congestive failure and outflow obstruction since a decrement in cardiac output caused by a decrease in cardiac preload produced by diuretics may further compromise patients' conditions.

Drug Category: Angiotensin converting enzyme inhibitors

ACE inhibitors are beneficial in all stages of chronic heart failure. Pharmacologic effects result in a decrease in systemic vascular resistance, reducing blood pressure, preload, and afterload. Dyspnea and exercise tolerance are improved. Blood pressure is determined by cardiac output and systemic resistance. When systemic resistance is decreased with afterload reduction, myocardial shortening and stroke volume improve; thus, cardiac output can be maintained at a lower heart rate with lower myocardial oxygen demand. ACE inhibitors decrease production of angiotensin II, a potent vasoconstrictor. As with the diuretics, pay special attention when administering to patients with outflow tract obstruction, since afterload is decreased.

Drug Category: Adrenergic agonist agents (inotropes)

These agents improve the hemodynamic status by increasing myocardial contractility and heart rate, resulting in increased cardiac output. They also increase peripheral resistance by causing vasoconstriction. Increased cardiac output and increased peripheral resistance lead to increased blood pressure.

Drug Category: Cyclic adenosine monophosphate (cAMP) phosphodiesterase inhibitors

Inotropic effect occurs by inhibiting cAMP phosphodiesterase that increases cellular levels of cAMP. It does not affect the sodium-potassium pump like digitalis. Vasodilatory activity is related to direct relaxation effect on vascular smooth muscle.

Drug Category: Antiarrhythmic agents

These agents alter the electrophysiologic mechanisms responsible for arrhythmia.

FOLLOW-UP

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Further Inpatient Care

• Discharge patients when they are stable.

Further Outpatient Care

• Provide close follow-up care to diagnose recurrence of malignant tumors after resection.
• Monitor ventricular function.
• Monitor medication doses and effects.

In/Out Patient Meds

• Medications, when indicated, include digitalis, afterload reduction agents, diuretics, antiarrhythmics, and anticoagulants.

Transfer

• Transfer to a facility with the necessary specialties and consultants.

Complications

• Complications include arrhythmia, CHF, thromboembolism, decrease in ventricular function, and seeding during surgical removal (metastasis).
• For complications related to specific types of cardiac tumors, see Background.

Prognosis

• Studies provide a wide spectrum of morbidity and mortality statistics.
• Survival rates depend on the type of tumor.
• • Benign tumors have a good prognosis unless they are associated with severe obstruction or intractable arrhythmias, which have a slightly higher risk of mortality.
  • For primary cardiac malignant tumors, the prognosis is invariably poor.
  • Metastatic neoplasms to the heart are more common than primary cardiac neoplasms; the prognosis is dependent on the histologic type of tumor, and survival rates are based on original pathology.
  • Certain tumors are known to recur even after resection.
  • Most children with cardiac tumors have an excellent prognosis and do not need any intervention.

MISCELLANEOUS

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Medical/Legal Pitfalls

• Failure to make the diagnosis in a timely manner

MULTIMEDIA

Section 10 of 11  

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Media file 1:  Echocardiographic parasternal long-axis view showing a large mass within the left ventricular cavity invading or connected to the anterior mitral valve leaflet. AO=Aorta, RV=Right ventricle, LA=Left atrium, Arrow=Left ventricular mass.
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Media type:  Image

Media file 2:  Echocardiographic subcostal view of a patient with a large mass within the left ventricular cavity invading or connected to the anterior mitral valve leaflet. Mass within the left ventricle fills most of the ventricular cavity. LA=Left atrium, RA=Right atrium, RV=Right ventricle, Arrow=Left ventricular mass.
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Media type:  Image

Media file 3:  Echocardiographic parasternal long-axis view demonstrating a rounded mass in the area of the right ventricular outflow tract. Mass was not causing any outflow obstruction. RV=Right ventricle, LV= Left ventricle, AO=Aorta, Arrow=Right ventricular mass.
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Media type:  Image

Media file 4:  Echocardiographic apical 5 chamber view of a 1 month old patient with several ventricular tumors filling the ventricular cavities in both sides. The patient was having seizures and diagnosed to have tuberous sclerosis. The tumor associated with this disease is rhabdomyoma (see text). RA= Right Atrium; Ao= Aorta; LA= Left Atrium; RV= Right Atrium; LV= Left Ventricle
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Media type:  Image

Media file 5:  Follow-up echocardiographic 4 chamber view from the same patient as in Picture #4. This echocardiogram was done 8 months after the previous view. Note that the tumors have changed in size and some of them have regressed totally, especially in the right ventricle. The arrows show the remaining areas of tumors seen in both ventricles.
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Media type:  CT

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
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• Differentials
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• Follow-up
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• References

• Butany J, Nair V, Naseemuddin A, et al. Cardiac tumours: diagnosis and management. Lancet Oncol. Apr 2005;6(4):219-28. [Medline].
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• Farooki ZQ, Ross RD, Paridon SM, et al. Spontaneous regression of cardiac rhabdomyoma. Am J Cardiol. Apr 15 1991;67(9):897-9. [Medline].
• Lam KY, Dickens P, Chan AC. Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med. Oct 1993;117(10):1027-31. [Medline].
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• Martinez Quesada M, Trujillo Berraquero F, Almendro Delia M, et al. [Cardiac hamartoma. Case report and literature review]. Rev Esp Cardiol. Apr 2005;58(4):450-2. [Medline].
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• Padalino MA, Basso C, Milanesi O, et al. Surgically treated primary cardiac tumors in early infancy and childhood. J Thorac Cardiovasc Surg. Jun 2005;129(6):1358-63. [Medline].
• Piazza N, Chughtai T, Toledano K, et al. Primary cardiac tumours: eighteen years of surgical experience on 21 patients. Can J Cardiol. Dec 2004;20(14):1443-8. [Medline].
• Rodriguez-Cruz E, Cintron-Maldonado RM, Bercu BA. Primary cardiac osteogenic sarcoma treated with heart transplantation. Bol Asoc Med PR. 1999;91 (7-12):98-99. [Medline].
• Rodriguez-Cruz E, Cintron-Maldonado RM, Forbes TJ. Treatment of primary cardiac malignancies with orthotopic heart transplantation. Bol Asoc Med P R. Apr-Aug 2000;92(4-8):65-71. [Medline].
• Takach TJ, Reul GJ, Ott DA, Cooley DA. Primary cardiac tumors in infants and children: immediate and long-term operative results. Ann Thorac Surg. Aug 1996;62(2):559-64. [Medline].
• Tatli S, Lipton MJ. CT for intracardiac thrombi and tumors. Int J Cardiovasc Imaging. Feb 2005;21(1):115-31. [Medline].
• Tazelaar HD, Locke TJ, McGregor CG. Pathology of surgically excised primary cardiac tumors. Mayo Clin Proc. Oct 1992;67(10):957-65. [Medline].
• Wu XM, Hu CZ, Li N, et al. [Relationship of serum level of creatine kinase of MB type to cardiac function of patients with advanced tumors and its prognostic value]. Ai Zheng. Apr 2005;24(4):506-8.abs. [Medline].

Article Last Updated: May 31, 2006