cardiac papillary fibroelastoma
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Epidemiology
- third most common benign cardiac neoplasm after cardiac myxomas & lipomas
Pathology
- fibroelastoma attached to the endocardium of a cardiac valve generally left-sided (case presentation[1] attachment to mitral valve)
- embolization may occur
- papillary structure predisposes embolization of neoplasm fragments or attached thrombus to the coronary, systemic, or cerebrovascular systems[1]
- most are small (1.2 cm x 0.9 cm average)
Radiology
- echocardiogram (may be incidental finding)
- may show a cardiac valve vegetation with a heterogeneous globular shape or a mobile frond-like appearance on the surface of the aortic valve or mitral valve
Differential diagnosis
- atrial myxoma
- larger than papillary fibroelastoma
- generally associated with obstructive features
- 75% arise from fossa ovalis of left atrium, not a cardiac valve
- fever, weight loss may be noted[1]
- a tumor 'plop' may be heard on auscultation
Management
- removal of the cardiac valve vegetation[1]
- papillary fibroelastoma with embolic symptoms are treated surgically
- large neoplasms (> 1 cm) often treated surgically even in the absence of symptoms[1]
- surveillance may be appropriate for smaller, asymptomatic lesions & those arising from right-sided valves
- insufficient evidence to support antiplatelet therapy or anticoagulation[1]
More general terms
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Medical Knowledge Self Assessment Program (MKSAP) 18, American College of Physicians, Philadelphia 2018
Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025 - ↑ Val-Bernal JF, Mayorga M, Garijo MF, Val D, Nistal JF. Cardiac papillary fibroelastoma: retrospective clinicopathologic study of 17 tumors with resection at a single institution and literature review. Pathol Res Pract. 2013 Apr;209(4):208-14. Review. PMID: https://pubmed.ncbi.nlm.nih.gov/23455367