AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

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

INTRODUCTION

Section 2 of 10  

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

Background

In general, primary tumors of the heart are rare. In autopsy studies, the overall prevalence ranges from 0.002-0.25%.

Although most tumors of the heart are benign, because of their malignant potential, the risks secondary to impaired cardiac function (eg, congestive heart failure, inflow/outflow tract obstruction), conduction system involvement, and/or peripheral embolism mandate prompt evaluation and definitive treatment.

The most common primary cardiac tumor is a myxoma. Other less common neoplastic tissue types occur; each has distinguishing characteristics that often aid in accurate preoperative diagnosis or diagnosis prior to death. A definitive diagnosis is important because some cardiac tumors can be malignant or, more commonly, can represent metastasis from a distant primary tumor.

Pathophysiology

Myxomas arise usually from the endocardium and range in size from 1-20 cm. The vast majority (75%) develop from the left atrium, with the remainder (15-20%) developing from the right atrium. They tend to develop from the fossa ovalis but can be found arising from anywhere in the atrium. Ventricular or valvular sites are rare.

Rhabdomyomas are intramural tumors that are typically smaller and most often involve the left (80%) or right (15%) ventricles.

Fibromas most commonly involve the intraventricular septum or left ventricular free wall. Less than 10% of reported cases have atrial or great vessel involvement. Unlike myxomas, tumor embolization is uncommon. Tumor growth can displace or directly involve mitral and aortic valves and result in hemodynamically significant valvular stenosis or regurgitation.

Symptoms typically are secondary to adverse effects on normal left ventricular geometry, filling, and ejection. Additionally, arrhythmias, particularly sudden death and abnormal atrioventricular conduction, are common because of tumor disruption of the nodal or septal conduction tissue.

Frequency

United States

Benign cardiac tumors are extremely rare. Of all primary cardiac tumors, 75% are histologically benign. Myxomas represent approximately 75% of benign tumors, while rhabdomyomas (5-10%) and fibromas (4-6%) occur less commonly.

Mortality/Morbidity

Systemic embolization is one of the most common causes of complications. It is typically a presenting symptom in 25-50% of cases. Cardiac tumor should be in the differential diagnosis when evaluating any cause of embolization. Rhabdomyomas often manifest early in life with inflow/outflow obstruction (ie, heart failure) or arrhythmias; resection is typically indicated.

Sex

No known sex predilection is recognized, although the rarity of the different benign cardiac tumors prevents accurate determination of a male-to-female ratio.

Age

Myxomas are the most common tumors in adults. However, rhabdomyoma is the most common tumor in children (second most common benign cardiac tumor overall). Fibromas are rare and typically occur in children.

CLINICAL

Section 3 of 10  

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

History

Any patient with an embolic complication or signs or symptoms of inflow or outflow obstruction (ie, left or right heart failure) should have an evaluation with cardiac tumor considered in the differential diagnosis.

• Heart failure
• • Chamber obliteration by tumor involvement or abnormal myocardial function and arrhythmias is secondary to intramyocardial tumor growth.
  • Tumor growth can cause signs and symptoms of left ventricular outflow tract obstruction.
  • Diastolic murmurs might indicate impaired valve function due to tumor compression or growth.
• Palpitations: Tumor involvement of the conduction system might cause palpitations or syncopal episodes.
• Sudden death: This has been reported in as many as 33% of cases.
• Syncope: Syncopal episodes might be related to associated arrhythmias.

Physical

• Neurologic findings
• • Focal deficits range from transient ischemic attacks to hemispheric stroke from cerebral embolism.
  • Embolic complications are extremely rare with fibromas but more common with myxomas.
• Pulmonary findings
• • Patients with impaired left ventricular filling or function might exhibit signs of congestive heart failure.
  • Signs include rales, shortness of breath, and cough.
• Abdominal findings: Right atrial or ventricular involvement can impair venous return and cause hepatosplenomegaly or ascites.
• Extremity examination findings: Heart failure can cause peripheral edema.

Causes

• Unknown etiology
• Causal association with Gorlin syndrome
• • Syndrome of multiple nevoid basal-cell carcinomas, cysts and fibrosarcomas of the jaws, and skeletal abnormalities
  • Multiple skin abnormalities, including milia, epidermoid cysts, chalazia, and comedones
  • Associated with medulloblastoma, meningioma, ovarian fibroma/fibrosarcoma, cardiac fibroma, fetal rhabdomyoma, and mesenteric lymphatic or chylous cysts
• Carney complex: Carney complex is a syndrome of myxoma, endocrine hyperfunction, and areas of skin pigmentation. Myxomas associated with this complex have a high risk of recurrence following resection.

DIFFERENTIALS

Section 4 of 10  

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

Other Problems to be Considered

Intracardiac thrombus

WORKUP

Section 5 of 10  

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

Lab Studies

• Blood cultures to evaluate the possibility of endocarditis

Imaging Studies

• Chest radiography
• • Radiographic findings are often unremarkable.
  • Enlargement of the cardiac silhouette or mediastinal widening might be present.
  • Focal cardiac calcifications are often suggestive and can be characteristic of a fibroma, especially in children.
• Echocardiography
• • It plays a fundamental role in the evaluation of patients with embolic complications, unexplained cardiac murmurs, or signs and symptoms of heart failure.
  • Cardiac ultrasonography represents the best diagnostic test for evaluating patients with history and/or physical examination findings suggestive of valvular dysfunction or the possibility of an intracardiac mass.
  • The ability to distinguish tissue characteristics, location, morphology, and mobility noninvasively, quickly, and without the use of ionizing radiation makes echocardiography the ideal diagnostic modality.
  • Equivocal transthoracic findings typically indicate the need for a transesophageal evaluation, during which the atria and great vessels might be better imaged.
  • Surgical intervention is often indicated based on possible echocardiographic findings, without the need for additional time-consuming, costly, or invasive studies.
• Magnetic resonance imaging
• • Once a cardiac mass is identified, MRI can be extremely beneficial in determining the extent of tumor involvement and cellular characteristics.
  • T1- and T2-weighted images often provide valuable clues regarding tissue characterization; spin-echo image intensities can help differentiate cardiac tumors.
  • MRI cannot help distinguish between benign and malignant tumors. A tissue diagnosis is required in order to distinguish between benign and malignant tumors.
  • Several small studies have suggested that MRI might be more sensitive and specific for primary cardiac tumors than echocardiography, but no large studies have demonstrated a benefit of MRI over echocardiography.
  • With equivocal echocardiographic findings, MRI might help differentiate an intracavitary tumor from a thrombus or hypertrophied papillary muscle.
• CT scanning
• • CT scanning is often performed during an evaluation for a possible thoracic malignancy, and findings might suggest a primary cardiac tumor.
  • CT scan findings might provide clues regarding tissue characterization, with central calcification suggestive of a cardiac fibroma.
  • Even with advances in ECG gating, cardiac CT scanning often offers little diagnostic value over a thorough echocardiographic evaluation.
• Angiography with cardiac catheterization
• • In patients with known intracardiac masses, this is relatively contraindicated because of the significant risk of catheter-induced tumor embolization.
  • In patients who possibly are at high risk for concomitant coronary disease and are undergoing surgical treatment, simple coronary angiography might be necessary.
  • If a mass is found as an incidental finding during angiography, take strict care to minimize disruption of the mass to avoid causing a systemic embolic complication.
  • Ventriculography might demonstrate filling defects suggestive of an intracavitary mass.

Other Tests

• Electrocardiography
• • Nonspecific changes and/or left-axis deviation might occur.
  • Repolarization abnormalities, similar to those found in persons with myocardial infarction or ischemia, are secondary to disruption of normal conduction patterns by electrically silent tumor or localized ischemia from tumor compression.

Histologic Findings

Myxomas are similar to pluripotent germ cells with acid mucopolysaccharide matrix. The cells appear spindle or polygonal shaped.

Microscopically, fibromas are a light-gray, complex interlacing of dense collagen with occasional fibroblasts. Cardiac fibromas are similar to fibromas found in other soft tissue locations, except for the lack of a distinct capsule. Fibromas can contain myocardial fibers, but these are believed to reflect entrapment of myoblasts during tumor growth rather than tissue invasion. Overall, growth tends to displace myocardial tissue rather than invade or destroy it.

TREATMENT

Section 6 of 10  

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

Medical Care

Medical management is not indicated unless symptoms are minimal, operative risks are prohibitive, or tumor size limits adequate resection. For patients who do not undergo surgical treatment, close consultation with a cardiac electrophysiologist is indicated. Because of the high risk of potentially lethal ventricular arrhythmias, prophylactic antiarrhythmics or a transvenous implanted cardioverter/defibrillator might be considered.

Rhabdomyomas have a high tendency to regress, therefore close follow-up with a pediatric cardiologist is needed. Indications for resection are usually for severe intracavitary obstruction.

Surgical Care

Complete surgical resection of benign masses typically is curative.

• Urgent treatment is recommended because the risk for lethal arrhythmias or embolization (with myxomas) is high.
• Cardiac transplantation has been reported in persons with large, unresectable tumors.

Consultations

• Cardiologist
• Cardiothoracic surgeon
• Oncologist if malignant disease is suspected

Activity

No restrictions on activity are necessary following elective resection (other than usual postoperative restrictures during the recovery phase).

MEDICATION

Section 7 of 10  

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

Medications are not indicated for primary treatment.

FOLLOW-UP

Section 8 of 10  

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

Further Outpatient Care

• Routine transthoracic echocardiographic evaluation is recommended every 2-5 years to evaluate for recurrence.
• According to American Heart Association guidelines, to minimize the risk of endocarditis, instruct the following patients to take prophylactic antibiotics:
• • Patients with residual postoperative valvular dysfunction
  • Patients with implanted prosthetic valves or artificial materials (used to repair postresection myocardial defects)
  • Patients with outflow tract obstruction

Complications

• Despite complete resection, patients are still at risk for sudden death due to damage to the conduction system.
• The role of prophylactic antiarrhythmics or implanted cardioverter/defibrillators is undefined.

Prognosis

• The prognosis is excellent, including disease-free survival, if the tumor is completely resected.
• Incomplete resections predispose patients to further tumor growth and recurrence of symptoms; risk is undefined.
• Although unproved, concern for recurrence, similar to that for fibromas found in other parts of the body, is warranted.

Patient Education

• For excellent patient education resources, please see eMedicine's Heart Center.

MISCELLANEOUS

Section 9 of 10  

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

Medical/Legal Pitfalls

• Regardless of etiology, patients with untreated intracardiac masses are at risk for embolic complications, lethal arrhythmias, and symptoms of heart failure.
• An inaccurate diagnosis (eg, thrombus) may subject patients to risky medical or surgical treatments (or a delay in surgical management).
• In the absence of a tissue diagnosis, a mass presumed benign can represent a primary or metastatic malignancy.

REFERENCES

Section 10 of 10

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

• Burke AP, Rosado-de-Christenson M, Templeton PA, Virmani R. Cardiac fibroma: clinicopathologic correlates and surgical treatment. J Thorac Cardio Surg. 1994;108(5):862-70. [Medline].
• Caralps JM, Montiel J, Reig J. Complete surgical excision of a huge left ventricular fibroma. J Thorac Cardiovasc Surg. Jun 2005;129(6):1444-5. [Medline].
• Centofanti P, DiRose E, Deorsola L, et al. Primary cardiac tumors: early and late results of surgical treatment in 91 patients. Ann Thorac Surg. 1999;68(4):1236-41. [Medline].
• Liddicoat J, Gillinov AM. Tumors of the Heart. In: Sabiston & Spencer: Surgery of the Chest. Vol 2. Philadelphia, Pa:. WB Saunders;2005:1741-1748.
• Lund JT, Ehman RL, Julsrud PR, et al. Cardiac masses: assessment by MR imaging. AJR Am J Roentgenol. Mar 1989;152(3):469-73. [Medline].
• Michler RE, Goldstein DJ. Treatment of cardiac tumors by orthotopic cardiac transplantation. Semin Oncol. 1997;24(5):534-9. [Medline].
• Muhler EG, Kienast W, Turniski-Harder V, von Bernuth G. Arrhythmias in infants and children with primary cardiac tumours. Euro Heart J. 1994;15(7):915-921.
• Padalino MA, Basso C, Milanesi O. Surgically treated primary cardiac tumors in early infancy and childhood. J Thorac Cardiovasc Surg. Jun 2005;129(6):1358-63. [Medline].
• Roberts WC. Primary and secondary neoplasms of the heart. Am J Cardiol. Sep 1 1997;80(5):671-82. [Medline].
• Salcedo EE, Cohen GI, White RD, Davison MB. Cardiac tumors: diagnosis and management. Curr Probl Cardiol. Feb 1992;17(2):73-137. [Medline].
• Tillmanns H. Clinical aspects of cardiac tumors. Thorac Cardiovasv Surg. 1990;38 (suppl 2):152-6.
• Williams DB, Danielson GK, McGoon DC, et al. Cardiac fibroma: long-term survival after excision. J Thorac Cardiovasc Surg. Aug 1982;84(2):230-6. [Medline].

Article Last Updated: Feb 2, 2007