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atrial myxoma

Most common primary cardiac tumor. Etiology: 1) sporadic 2) associated with NAME or LAMB syndrome Epidemiology: 1) sporadic form a) 39-82 years; mean age: 56 years b) female/male ratio 2.7/1 2) associated with NAME syndrome or LAMB syndrome a) 10-56 years; mean age: 25 years b) female/male ratio 1.8/1 Pathology: 1) location: a) left atrium 75% b) right atrium 18% c) most arise from atrial septum (fossa ovalis) 2) most myxomas have a short stalk, are gelatinous & friable, & tend to embolize 3) left atrial tumors prolapse into the mitral valve & cause mitral stenosis 4) occasionally calcify 5) systemic emboli occur in 30-60% of patients with left-sided myxoma, frequently to brain & lower extremities 6) sporadic form: 99% have single myxoma 7) associated with NAME syndrome or LAMB syndrome a) 50% have multiple myxomas b) 57% with non-cardiac tumors c) 30% with associated endocrine tumor d) 13% with ventricular myxoma Genetics: 1) associated with NAME syndrome or LAMB syndrome - 14% autosomal dominant 2) associated with defects in PRKAR1A gene (autosomal recessive) Clinical manifestations: 1) obstruction to blood flow 2) embolization 3) systemic effects a) fatigue/malaise b) fever c) progressive dyspnea d) orthopnea e) weight loss f) arthralgia g) clubbing 4) symptoms occur with changes in body position 5) an early diastolic sound or tumor plop may be heard 6) soft-mid diastolic murmur best heard at the cardiac apex - may change with changes in body position 7) autosomal dominant form (NAME syndrome or LAMB syndrome) - 68% with associated freckles 8) cardiac arrhythmias are uncommon [5] Laboratory: 1) histologic examination of embolized material 2) with systemic manifestations a) ESR: elevated ESR b) complete blood count: leukocytosis c) protein electrophoresis: hypergammaglobulinemia (IgG) Radiology: 1) calcified myxomas may be visible on chest X-ray 2) echocardiogram Complications: - systemic embolism, embolic stroke - sudden death [2] Differential diagnosis: - diastolic murmur similar to mitral stenosis - mitral valve endocarditis associated with mitral regurgitation - marantic endocarditis - signs & symptoms of systemic embolization - rarely murmurs Management: 1) surgery 2) left atrial myxoma should be removed even if patient is asymptomatic to avoid systemic embolism, embolic stroke [2] 3) myxomas may be recurrent, thus periodic long-term follow-up is indicated [2]

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LAMB syndrome NAME syndrome

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cardiac myxoma

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 56
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022.
  3. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1102
  4. Elbardissi AW, Dearani JA, Daly RC et al Embolic potential of cardiac tumors and outcome after resection: a case-control study. Stroke. 2009 Jan;40(1):156-62 PMID: 18948602
  5. Ali YS Fast Five Quiz: Heart Palpitations. Medscape. October 10, 2022 https://reference.medscape.com/viewarticle/981860