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AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Mary C Mancini is a member of the following medical societies: American Heart Association, American Medical Association, American Thoracic Society, Association for Academic Surgery, Association for Surgical Education, International College of Surgeons, International Society for Heart and Lung Transplantation, New York Academy of Sciences, Phi Beta Kappa, and Southern Thoracic Surgical Association
Editors: Clarence Sarkodee-Adoo, MD, Consulting Staff, Department of Bone Marrow Transplantation, City of Hope Samaritan BMT Program; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Benjamin Movsas, MD, Vice-Chairman, Department of Radiation Oncology, Fox Chase Cancer Center; Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems; 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
Author and Editor Disclosure
Synonyms and related 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
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 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.
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.
Carney Complex
Lab Studies
Erythrocyte sedimentation rate (ESR) is a nonspecific test for which results may be elevated in the presence of a cardiac tumor.
Imaging Studies
- Chest radiograph may demonstrate an enlarged cardiac silhouette or atrial enlargement.
- MRI of the chest will demonstrate intracardiac pathology in the form of a mass.14, 15
- Transesophageal echocardiography (TEE) is the test of choice.16 TEE delineates the position of the tumor and details the integrity or involvement of the septal and valvular structures. (Seventy-five percent of cardiac myxomas arise from the fossa ovalis on the left atrial side.17)
- Perform fetal ultrasonography if the diagnosis is suspected in utero.
- Cardiac catheterization is necessary only if other processes are strongly suspected.
- Perform metaiodobenzylguanidine (MIBG) radionuclide scan for suspected paraganglioma. This scan uses a radioactive guanidine that is taken up preferentially by neuroendocrine cells.
Other Tests
ECG is ordered to detect potential arrhythmias that may be associated with tumors. These arrhythmias include atrial fibrillation, paroxysmal atrial tachycardia, and ventricular arrhythmias.
Histologic Findings
Myxomas are gelatinous in nature with rests of spindle cells (see Media files 1-2).18 If DNA tetraploidy is present, suspect malignancy. Fibroelastomas are composed of endocardium, fibrous tissue, elastic fibers, and smooth muscle cells arranged in a central stock of collagen and a covering of hyperplastic endothelial cells.
Staging
No particular staging exists for cardiac tumors.
Medical Care
For those tumors presenting with cardiac arrhythmias, standard medical management of the arrhythmia is warranted until the diagnosis is established and definitive therapy undertaken. In the case of paragangliomas, treat hypertension with beta-blockade until resection is undertaken.
Surgical Care
When possible, treatment of choice for cardiac tumors is complete resection.19 In patients whose tumors are determined to be malignant, adjuvant therapy (ie, chemotherapy, radiation) after resection did not affect the outcome.5 Cardiac transplantation has been used for benign and malignant tumors.20 Series using this therapy have been too small to use in predicting outcomes.
For patients who present with paraganglioma, hypertension, or arrhythmias, beta-blockade is the medical treatment of choice until surgical intervention can be undertaken.
Drug Category: Beta-blockers
This category of drugs has the potential to suppress ventricular ectopy due to ischemia or excess catecholamines. In the setting of myocardial ischemia, beta-blockers have anti-arrhythmic properties and reduce myocardial oxygen demand secondary to elevations in heart rate and inotropy.
| Drug Name | Metoprolol (Lopressor) |
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| Description | Selective beta1 adrenergic receptor blocker that decreases automaticity of contractions. During IV administration, carefully monitor BP, heart rate, and ECG. |
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| Adult Dose | 12.5-50 mg PO tid; increase at 1-wk intervals prn to total of 450 mg/d if necessary |
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| Pediatric Dose | 1-5 mg/kg/d PO divided bid |
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| Contraindications | Documented hypersensitivity; uncompensated CHF; bradycardia; asthma; cardiogenic shock; AV conduction abnormalities; chronic obstructive lung disease |
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| Interactions | Aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease bioavailability and plasma levels, possibly resulting in decreased pharmacologic effects; sparfloxacin, phenothiazines, astemizole, calcium channel blockers, quinidine, flecainide, and contraceptives may increase toxicity; may increase toxicity of digoxin, flecainide, clonidine, epinephrine, nifedipine, prazosin, verapamil, and lidocaine |
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| Pregnancy | B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
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| Precautions | Beta-adrenergic blockade may reduce signs and symptoms of acute hypoglycemia and may decrease clinical signs of hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; monitor patient closely and withdraw drug slowly; during IV administration, carefully monitor BP, heart rate, and ECG |
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| Drug Name | Carvedilol (Coreg) |
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| Description | Nonselective beta-adrenoreceptor blocker with alpha-adrenergic blocking activity of equal potency. No intrinsic sympathomimetic activity has been documented. |
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| Adult Dose | 3.125-12.5 mg PO bid |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; bradycardia; hypotension; severe cardiomyopathy; second- or third-degree heart block; chronic obstructive lung disease; symptomatic hepatic disease |
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| Interactions | Interaction with other anti-arrhythmics and antihypertensives may potentiate clinical effect; may increase blood levels of cyclosporine and digoxin; may diminish hyperglycemic action of glucagon; may alter response to hypoglycemic agents (ie, sulfonylureas); fluoxetine and paroxetine may increase blood level; rifampin may decrease blood level; NSAIDs may blunt antihypertensive effect |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
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| Precautions | Monitor BP and pulse before taking; use with caution in CHF with slowed AV conduction, peripheral vascular disease, hyperthyroidism, or diabetes mellitus; discontinue if hepatic dysfunction occurs; avoid abrupt withdrawal; discontinue over 1-2 wk |
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| Drug Name | Atenolol (Tenormin) |
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| Description | Selectively blocks beta1 receptors with little or no effect on beta2 receptors. |
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| Adult Dose | 25-50 mg PO bid |
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| Pediatric Dose | 1-2 mg/kg PO qd |
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| Contraindications | Documented hypersensitivity; CHF; pulmonary edema; cardiogenic shock; AV conduction abnormalities; heart block (without pacemaker) |
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| Interactions | Aluminum salts, barbiturates, calcium salts, cholestyramine, NSAIDs, penicillins, and rifampin may decrease effects; haloperidol, hydralazine, loop diuretics, and MAOIs may increase toxicity |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
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| Precautions | Monitor BP and pulse before taking; beta-adrenergic blockade may reduce symptoms of acute hypoglycemia and mask signs of hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism and cause thyroid storm; monitor patients closely and withdraw drug slowly; during IV administration, carefully monitor BP, heart rate, and ECG |
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Further Outpatient Care
Annually monitor patients who have undergone resection for cardiac tumors with echocardiography in order to assess for recurrences. With the more aggressive malignant tumors, follow up every 3-6 months.
Complications
Complications encountered after resection of cardiac tumors can include valvular insufficiency, complete heart block requiring pacemaker insertion, atrial and ventricular arrhythmias, and stroke.
Prognosis
Prognosis for benign tumors is excellent, matching that of the healthy population. Malignant tumors carry a dismal prognosis even in the face of complete resection. An occasional sarcoma can be resected completely, resulting in long-term survival.
Medical/Legal Pitfalls
- Although primary cardiac tumors are uncommon, the diagnosis must be entertained in any patient presenting with peripheral arterial embolization.
- In order to avoid missing the diagnosis, always send embolectomy specimens for pathologic examination, and carefully review the results of the examination.21
- If operative intervention is warranted once the diagnosis of an intracardiac tumor is made, undertake the procedure expeditiously. Delaying an intervention can invite complications from embolization of the tumor, including limb loss and stroke.
- Carefully instruct the patient and family that in spite of resection, these tumors might recur even though they are not malignant in most cases. In this regard, careful education will help eliminate the question of incomplete resection if recurrences arise.
Special Concerns
- Patient and family counseling about the disease process is important.
- For benign tumors cured by complete resection, the discussion is fairly straightforward. Explain the operative risk, complications, and alternatives, as with any cardiac operation.
- With rhabdomyomas, the common association with tuberous sclerosis requires careful discussion of the ramifications of this underlying condition. In addition, the presence of these tumors does not mandate resection unless associated with severe cardiac symptoms.
- Mesotheliomas of pericardial origin are usually malignant,22 while those of the AV node are not malignant.23
- The degree of malignancy of pheochromocytomas often cannot be defined histologically but is determined by degree of invasion and by development of metastases. Thus, some uncertainty is introduced into the discussion with the patient with this type of tumor.
| Media file 1:
Low-power photomicrograph of cardiac myxoma (hematoxylin and eosin stain). |
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Media type: Photo
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| Media file 2:
High-power photomicrograph showing the histology of cardiac myxoma (hematoxylin and eosin stain). Note the dark staining polygonal cells characteristic of the tumor. |
 | View Full Size Image | |
Media type: Photo
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- Attar S, Lee YC, Singleton R, et al. Cardiac myxoma. Ann Thorac Surg. May 1980;29(5):397-405. [Medline].
- Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer. Jan 15 1992;69(2):387-95. [Medline].
- Butany J, Nair V, Naseemuddin A, et al. Cardiac tumours: diagnosis and management. Lancet Oncol. Apr 2005;6(4):219-28. [Medline].
- O'Neil MB Jr, Grehl TM, Hurley EJ. Cardial myxomas: a clinical diagnostic challenge. Am J Surg. Jul 1979;138(1):68-76. [Medline].
- Llombart-Cussac A, Pivot X, Contesso G, et al. Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience. Br J Cancer. Dec 1998;78(12):1624-8. [Medline].
- Basu S, Folliguet T, Anselmo M, et al. Lipomatous hypertrophy of the interatrial septum. Cardiovasc Surg. Apr 1994;2(2):229-31. [Medline].
- Smythe JF, Dyck JD, Smallhorn JF, et al. Natural history of cardiac rhabdomyoma in infancy and childhood. Am J Cardiol. Nov 15 1990;66(17):1247-9. [Medline].
- Shahian DM, Labib SB, Chang G. Cardiac papillary fibroelastoma. Ann Thorac Surg. Feb 1995;59(2):538-41. [Medline].
- Burke AP, Virmani R. Cardiac rhabdomyoma: a clinicopathologic study. Mod Pathol. Jan 1991;4(1):70-4. [Medline].
- Burke AP, Rosado-de-Christenson M, Templeton PA, et al. Cardiac fibroma: clinicopathologic correlates and surgical treatment. J Thorac Cardiovasc Surg. Nov 1994;108(5):862-70. [Medline].
- Brizard C, Latremouille C, Jebara VA, et al. Cardiac hemangiomas. Ann Thorac Surg. Aug 1993;56(2):390-4. [Medline].
- Garson A, Smith RT Jr, Moak JP, et al. Incessant ventricular tachycardia in infants: myocardial hamartomas and surgical cure. J Am Coll Cardiol. Sep 1987;10(3):619-26. [Medline].
- Jebara VA, Uva MS, Farge A, et al. Cardiac pheochromocytomas. Ann Thorac Surg. Feb 1992;53(2):356-61. [Medline].
- 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].
- Sparrow PJ, Kurian JB, Jones TR, et al. MR imaging of cardiac tumors. Radiographics. Sep-Oct 2005;25(5):1255-76. [Medline].
- Reeder GS, Khandheria BK, Seward JB, et al. Transesophageal echocardiography and cardiac masses. Mayo Clin Proc. Nov 1991;66(11):1101-9. [Medline].
- Read RC, White HJ, Murphy ML, et al. The malignant potentiality of left atrial myxoma. J Thorac Cardiovasc Surg. Dec 1974;68(6):857-68. [Medline].
- Ferrans VJ, Roberts WC. Structural features of cardiac myxomas. Histology, histochemistry, and electron microscopy. Hum Pathol. Mar 1973;4(1):111-46. [Medline].
- Murphy MC, Sweeney MS, Putnam JB Jr, et al. Surgical treatment of cardiac tumors: a 25-year experience. Ann Thorac Surg. Apr 1990;49(4):612-7; discussion 617-8. [Medline].
- Michler RE, Goldstein DJ. Treatment of cardiac tumors by orthotopic cardiac transplantation. Semin Oncol. Oct 1997;24(5):534-9. [Medline].
- Basso C, Valente M, Poletti A, et al. Surgical pathology of primary cardiac and pericardial tumors. Eur J Cardiothorac Surg. Nov 1997;12(5):730-7; discussion 737-8. [Medline].
- Thomason R, Schlegel W, Lucca M, et al. Primary malignant mesothelioma of the pericardium. Case report and literature review. Tex Heart Inst J. 1994;21(2):170-4. [Medline].
- Balasundaram S, Halees SA, Duran C. Mesothelioma of the atrioventricular node: first successful follow-up after excision. Eur Heart J. May 1992;13(5):718-9. [Medline].
- Bhan A, Mehrotra R, Choudhary SK, et al. Surgical experience with intracardiac myxomas: long-term follow-up. Ann Thorac Surg. Sep 1998;66(3):810-3. [Medline].
- Butany J, Leong SW, Carmichael K, et al. A 30-year analysis of cardiac neoplasms at autopsy. Can J Cardiol. Jun 2005;21(8):675-80. [Medline].
- Jelic J, Milicic D, Alfirevic I, et al. Cardiac myxoma: diagnostic approach, surgical treatment and follow-up. A twenty years experience. J Cardiovasc Surg (Torino). Dec 1996;37(6 Suppl 1):113-7. [Medline].
- La Francesca S, Gregoric ID, Cohn WE, et al. Successful resection of a primary left ventricular schwannoma. Ann Thorac Surg. May 2007;83(5):1881-2. [Medline].
- McAllister HA Jr. Primary tumors and cysts of the heart and pericardium. Curr Probl Cardiol. May 1979;4(2):1-51. [Medline].
- Muzzi L, Davoli G, Specchia L, et al. Primary hemangioma of the mitral valve: an unusual presentation. J Heart Valve Dis. Mar 2007;16(2):209-11. [Medline].
- Nonami A, Takenaka K, Kamezaki K, et al. Successful treatment of primary cardiac lymphoma by rituximab-CHOP and high-dose chemotherapy with autologous peripheral blood stem cell transplantation. Int J Hematol. Apr 2007;85(3):264-6. [Medline].
- 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].
- Paul S, Ramanathan T, Cohen DM, et al. Primary Ewing sarcoma invading the heart: resection and reconstruction. J Thorac Cardiovasc Surg. Jun 2007;133(6):1667-9. [Medline].
- Peregud-Pogorzelska M, Kazmierczak J, Wojtarowicz A. Intracavitary mass as the initial manifestation of primary pericardial mesothelioma: a case report. Angiology. Apr-May 2007;58(2):255-8. [Medline].
- Putnam JB Jr, Sweeney MS, Colon R, et al. Primary cardiac sarcomas. Ann Thorac Surg. Jun 1991;51(6):906-10. [Medline].
- Reynen K. Cardiac myxomas. N Engl J Med. Dec 14 1995;333(24):1610-7. [Medline].
- Silverman NA. Primary cardiac tumors. Ann Surg. Feb 1980;191(2):127-38. [Medline].
- Stern MJ, Cohen MV, Fish B, et al. Clinical presentation and non-invasive diagnosis of right heart masses. Br Heart J. Nov 1981;46(5):552-8. [Medline].
- Tatli S, Lipton MJ. CT for intracardiac thrombi and tumors. Int J Cardiovasc Imaging. Feb 2005;21(1):115-31. [Medline].
- Wold LE, Lie JT. Cardiac myxomas: a clinicopathologic profile. Am J Pathol. Oct 1980;101(1):219-40. [Medline].
Cardiac Neoplasms, Primary excerpt Article Last Updated: Aug 7, 2008
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