eMedicine Specialties > Oncology > Carcinomas of the Lung and Other Intrathoracic Carcinomas

Cardiac Neoplasms, Primary

Author: Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Contributor Information and Disclosures

Updated: Aug 7, 2008

Introduction

Background

The most common primary cardiac tumor is the atrial myxoma, which accounts for 40-50% of all these neoplasms.1 The remainder of the pathological spectrum includes benign and malignant cell types. Although the overall incidence of primary cardiac neoplasms is low (0.0001-0.5% in autopsy series), these cardiac tumors provide unique diagnostic and therapeutic challenges.2,3,4


Low-power photomicrograph of cardiac myxoma (hema...

Low-power photomicrograph of cardiac myxoma (hematoxylin and eosin stain).

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Low-power photomicrograph of cardiac myxoma (hema...

Low-power photomicrograph of cardiac myxoma (hematoxylin and eosin stain).


High-power photomicrograph showing the histology ...

High-power photomicrograph showing the histology of cardiac myxoma (hematoxylin and eosin stain). Note the dark staining polygonal cells characteristic of the tumor.

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High-power photomicrograph showing the histology ...

High-power photomicrograph showing the histology of cardiac myxoma (hematoxylin and eosin stain). Note the dark staining polygonal cells characteristic of the tumor.


The clinical symptoms caused by cardiac tumors are generally secondary to their mass effect, local invasion, embolization, or constitutional symptoms. An intracardiac tumor mass may obstruct blood flow, compromise valve function, or induce neurological catastrophe secondary to tumor embolization. The location of the tumor determines the type of symptoms produced, which can include syncope, angina, dyspnea, edema, ascites, depression of pump function, cardiomyopathy, and pulmonary hypertension. Some tumors produce no symptoms and are found incidentally as a consequence of secondary symptoms such as stroke or evidence of peripheral embolization.

Most benign tumors can be resected completely with excellent outcomes. Consider heart transplantation in those instances in which the benign tumor is too large to resect. Resection is the treatment of choice for malignant cardiac tumors; however, long-term results are dismal, even with the addition of adjuvant therapy.5

Pathophysiology

Several types of tumors can arise in the heart, depending upon the tissues and structures involved. Primary neoplasms may arise from endocardium, valvular structures, primitive tissue rests, and the conduction system. Secondary or metastatic neoplasms arise from hematologic spread of the originating tissue. The physiologic derangements induced by the tumor are dependent upon the location of the mass and the tissue from which it arises.

Frequency

United States

Incidence depends upon tumor cell type. Overall incidence of the disease is 0.0001-0.5% in autopsy series. Myxomas account for 40-50% of primary cardiac tumors in patients aged 30-60 years.

International

Rates of cardiac tumors in other countries parallel rates in the United States.

Mortality/Morbidity

  • Myxomas: The mortality rate of patients after myxoma removal does not differ significantly from that of the general population.
  • Rhabdomyoma, fibroma, fibroelastoma, hemangioma, lipoma, teratoma, and hamartoma: These are benign tumors, and mortality rates in patients with these tumors do not differ significantly from that of the general population.
  • Malignant tumors: These tumors are generally sarcomatous in nature. In spite of resections (complete or incomplete), median survival duration ranges from 10-24 months.

Race

Primary cardiac tumors appear to occur equally in all races, unless their presence is connected directly with underlying genetic disorders that demonstrate a race predilection.

Sex

The frequencies of these tumors in males and females are the same.

Age

Cardiac tumors can occur in all age groups.

  • Cardiac myxoma, lipomatous septal hypertrophy,6 paraganglioma, and sarcoma tumors occur primarily in adults.
  • Rhabdomyoma, fibroma, teratoma, hamartoma, and Purkinje tumors occur primarily in children.7
  • Papillary fibroelastoma,8 hemangioma, and lipoma occur in all age groups.

Clinical

History

Myxomas are often asymptomatic, but these tumors can produce symptoms by releasing substances that lead to inflammatory signs, including fever, tachycardia, and tachypnea. Myxomas also present as acute vascular insufficiency of the extremities from embolization. If myxomas are large, they will cause intracardiac obstruction manifested as dyspnea, syncope, or congestive heart failure.

  • Rhabdomyoma arises from the ventricles of atrioventricular (AV) valves and regress spontaneously. It presents with atrial arrhythmias and heart block. Approximately 80% of rhabdomyomas are associated with tuberous sclerosis. Although most rhabdomyomas are small, large ones may cause intracardiac obstruction.9
  • Fibroma,10 hemangioma,11 and lipoma are accompanied by signs and symptoms that are attributable to the mass effect of the tumor. Depending upon the location, these tumors may obstruct the right or left ventricular outflow, resulting in dyspnea, hepatic congestion, peripheral edema, and syncope. Arrhythmias may occur when the tumor is located at the ventricular septum.
  • Papillary fibroelastomas typically arise from the aortic or mitral valves; they may be asymptomatic or may be detected secondary to an embolic event. These typically are found incidentally on echocardiography.
  • Lipomatous septal hypertrophy and hamartoma or Purkinje tumors generally present with arrhythmias. In the former case, atrial arrhythmias and heart block are common. In the latter case, incessant ventricular tachycardia in an infant is indicative of the presence of a tumor.12
  • Teratoma and paraganglioma tumors directly compress the heart, causing tamponade symptoms (ie, hypotension, fatigue, dyspnea). These tumors may present with superior vena cava obstructive symptoms. Paraganglioma also may produce systemic symptoms such as hypertension, headache, flushing, and palpitations.
  • Of pheochromocytomas occurring as a cardiac tumor, 50% secrete catecholamines, producing the characteristic symptoms of hypertension, arrhythmias, anxiety, palpitations, and perspiration.13
  • For sarcomatous tumors, pain suggests malignancy. Dyspnea, pericardial effusions, right heart failure, and syncope are common.

Physical

Often, examination of patients with cardiac tumors yields no abnormal physical findings unless the tumor is large or produces substances that may cause secondary symptoms or findings.

  • Patients with cardiac myxoma may present with an acutely ischemic extremity and absent pulses due to embolization.
  • In a similar fashion, tumor emboli may produce neurological signs of stroke.
  • Patients with large prolapsing myxomas may have an early diagnostic sound termed the tumor plop. This sound results from the impact of the tumor against the endocardial wall. If the tumor is present in the left ventricle and compromises the mitral valve, the murmur of mitral stenosis may be heard.
  • In a similar fashion, masses producing ventricular compromise may manifest signs such as peripheral edema, hepatic enlargement, cyanosis, or clubbing.
  • Tumors that compress the heart may be manifested by the classic physical findings of cardiac tamponade, ie, jugular venous distention, muffled heart tones, and pulsus paradoxus.
  • Notably, no classic physical examination finding defines the presence of a cardiac tumor in a patient.

Causes

No distinct cause of cardiac tumors is known.

More on Cardiac Neoplasms, Primary

Overview: Cardiac Neoplasms, Primary
Differential Diagnoses & Workup: Cardiac Neoplasms, Primary
Treatment & Medication: Cardiac Neoplasms, Primary
Follow-up: Cardiac Neoplasms, Primary
Multimedia: Cardiac Neoplasms, Primary
References
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Further Reading

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Keywords

primary cardiac neoplasms, cardiac tumor, heart tumor, heart cancer, myxoma, Carney complex, atrial myxoma, intracardiac tumor mass, primary neoplasm, rhabdomyoma, fibroma, fibroelastoma, hemangioma, lipoma, teratoma, hamartoma, malignant tumor, benign tumor

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Contributor Information and Disclosures

Author

Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Mary C Mancini, MD, PhD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons, and Southern Surgical Association
Disclosure: Nothing to disclose.

Medical Editor

Clarence Sarkodee-Adoo, MD, Consulting Staff, Department of Bone Marrow Transplantation, City of Hope Samaritan BMT Program
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Benjamin Movsas, MD, Vice-Chairman, Department of Radiation Oncology, Fox Chase Cancer Center
Benjamin Movsas, MD is a member of the following medical societies: American College of Radiology, American Radium Society, and American Society for Therapeutic Radiology and Oncology
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Jules E Harris, MD, Clinical Professor of Medicine, Division of Hematology/Medical Oncology, Department of Internal Medicine, University of Arizona College of Medicine at Tucson; Consulting Staff, Arizona Cancer Center
Jules E Harris, MD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Immunologists, American Society of Hematology, and Central Society for Clinical Research
Disclosure: GlobeImmune Salary Consulting; Amplimed Consulting fee Consulting

 
 
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