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Benign Cardiac Tumors

  • Author: Dale K Mueller, MD; Chief Editor: Karlheinz Peter, MD, PhD  more...
 
Updated: Jan 01, 2015
 

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.

It has been reported that patients with benign cardiac tumors are at increased risk of first ischemic stroke, particularly patients younger than 50 years.[1]

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.

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Pathophysiology

Myxomas arise usually from the endocardium and range in size from 1-20 cm. The vast majority (86%) develop from the left atrium, with the remainder developing from the right atrium.[2] 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 are typically 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. Benign cardiac tumors can also present without symptoms as incidental findings found on cardiac studies investigating other pathology.

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Epidemiology

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

The prognosis is excellent, including disease-free survival, if the tumor is completely resected.[11] 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.

Systemic embolization is the most common causes of complications. It is typically a presenting symptom in 25-50% of cases. Embolization can occur to any end organ including the brain, lower extremities, kidneys, and heart (coronary artery) with subsequent ischemia and possible infarction. 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 arrhythmia and resection is typically indicated.

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.

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.

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Contributor Information and Disclosures
Author

Dale K Mueller, MD Co-Medical Director of Thoracic Center of Excellence, Chairman, Department of Cardiovascular Medicine and Surgery, OSF Saint Francis Medical Center; Cardiovascular and Thoracic Surgeon, HeartCare Midwest, Ltd, A Subsidiary of OSF Saint Francis Medical Center; Section Chief, Department of Surgery, University of Illinois at Peoria College of Medicine

Dale K Mueller, MD is a member of the following medical societies: American College of Chest Physicians, American College of Surgeons, American Medical Association, Chicago Medical Society, Illinois State Medical Society, International Society for Heart and Lung Transplantation, Society of Thoracic Surgeons, Rush Surgical Society

Disclosure: Received consulting fee from Provation Medical for writing.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Steven J Compton, MD, FACC, FACP, FHRS Director of Cardiac Electrophysiology, Alaska Heart Institute, Providence and Alaska Regional Hospitals

Steven J Compton, MD, FACC, FACP, FHRS is a member of the following medical societies: American College of Physicians, American Heart Association, American Medical Association, Heart Rhythm Society, Alaska State Medical Association, American College of Cardiology

Disclosure: Nothing to disclose.

Chief Editor

Karlheinz Peter, MD, PhD Professor of Medicine, Monash University; Head of Centre of Thrombosis and Myocardial Infarction, Head of Division of Atherothrombosis and Vascular Biology, Associate Director, Baker Heart Research Institute; Interventional Cardiologist, The Alfred Hospital, Australia

Karlheinz Peter, MD, PhD is a member of the following medical societies: American Heart Association, German Cardiac Society, Cardiac Society of Australia and New Zealand

Disclosure: Nothing to disclose.

Additional Contributors

Russell F Kelly, MD Assistant Professor, Department of Internal Medicine, Rush Medical College; Chairman of Adult Cardiology and Director of the Fellowship Program, Cook County Hospital

Russell F Kelly, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases gratefully acknowledge the contributions of previous authors Michael S Firstenberg, MD, and James D Thomas, MD, to the development and writing of this article.

References
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  8. Reardon MJ, Malaisrie SC, Walkes JC, et al. Cardiac autotransplantation for primary cardiac tumors. Ann Thorac Surg. 2006 Aug. 82(2):645-50. [Medline].

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  10. Michler RE, Goldstein DJ. Treatment of cardiac tumors by orthotopic cardiac transplantation. Semin Oncol. 1997. 24(5):534-9. [Medline].

  11. Williams DB, Danielson GK, McGoon DC, et al. Cardiac fibroma: long-term survival after excision. J Thorac Cardiovasc Surg. 1982 Aug. 84(2):230-6. [Medline].

 
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