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  • • Most common cardiac neoplasm is a metastatic lesion (carcinoma of lung or breast, sarcoma, melanoma)

    • 75% of primary cardiac neoplasms are benign (myxoma, rhabdomyoma)

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Myxoma

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  • • 75% of benign primary cardiac tumors

    • Appearance ranges from smooth, round, firm encapsulated mass to loose conglomeration of gelatinous material

    • Most attached to fossa ovalis of left atrial septum; some may occur in right atrium or ventricles

    • Histologically, contains various mesenchymal cells

    • -Abnormal DNA ploidy may correlate with recurrence

    • Papillary fronds attached to aortic valve associated with cerebral and coronary embolization

    • Fibromas occur in pediatric patients, slowly invading conduction system

    • -Can cause sudden death from arrhythmias

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Epidemiology

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  • • Primary tumors of heart are rare

    • -0.002-0.3% of autopsies

    • Occur in any age

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Symptoms and Signs

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  • • Presentation depends on type and location of tumor

    Malignant tumors: Rapidly progressive congestive heart failure from valvular or myocardial infiltration

    Myxoma: Fever, weight loss, anemia, systemic embolization

    • Mitral stenosis can occur from tumor causing characteristic early diastolic sound "tumor plop"

    • Fibromas can cause sudden death from arrhythmias

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Laboratory Findings

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  • Myxoma: Abnormal ESR, gamma globulin, liver aminotransferases

    • Anemia, thrombocytopenia common in many tumors

    Transesophageal echocardiography: Procedure of choice

    • MRI and CT may be helpful in infiltrative lesions

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  • • Evaluate for other sites of tumor

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  • • Physical exam

    • Echocardiography, possibly transesophageal

    • MRI for invasive lesions

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  • Most benign lesions: Resectable/curable

    Myxomas

    • -Cardiopulmonary bypass required

      -Resect tumor and rim of normal tissue around attachment stalk

    • Surgery for cardiac sarcomas and metastatic lesions is usually for diagnosis; occasionally palliative

    • Orthotopic heart transplantation (rarely)

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Surgery

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Indications

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  • • Suspected myxoma, consider repair

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Complications

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  • • Embolization during tumor manipulation

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Prognosis

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  • • Operative mortality < 1%

    • Long-term survival for malignant cardiac lesions remains poor

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Prevention

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  • • Resect rim of normal tissue around attachment stalk to prevent recurrence of myxomas

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References

Araoz PA et al. CT and MR imaging of primary cardiac malignancies. Radiographics. 1999;19:1421.  [PubMed: 10555666]
Lobo A et al. Intracardiac masses detected by echocardiography: case presentation and review of the literature. Clin Cardiol. 2000;23:702.  [PubMed: 11016023]
Shapiro LM. Cardiac tumours: diagnosis and management. Heart. 2001;85:218.  [PubMed: 11156679]
Gavrielatos G. et al. Large left atrial myxoma presented as fever of unknown origin: a challenging diagnosis and a review of the literature. Cardiovascular Pathology. 2007;16(6):365-7.  [PubMed: 18005878]

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