Type:
Educational Exhibit
Keywords:
Not applicable, Neoplasia, Diagnostic procedure, Contrast agent-intravenous, MR, Echocardiography, CT, Cardiovascular system, Cardiac
Authors:
A. Perja, R. Rancea, A. Molnar, S. Encică, S. Manole; Cluj-Napoca/RO
DOI:
10.26044/ecr2020/C-00458
Background
Epidemiology
- rare benign primary cardiac tumor
- the most common tumor of the valves
- the second most common cardiac benign tumor, following myxomas [1,2]
- all age groups, most frequently between the 4th and the 8th decade of life [1-3]
- equal gender prevalence [1,2], although some studies found either a slight male [3] or female [4,5] predilection
- usually a solitary tumor, may be multiple [6]
Location
- endocardium
- typically cardiac valves, especially in the left heart, aortic valve the most frequently affected [1-3,7]
- non-valvular sites of origin: left ventricle, ostium of coronary arteries, atrial appendages, atrial septum, ventricular septum, ventricular outflow tracts, right atrium, Eustachian valve, Chiari network [2,3]
Pathology
Macroscopically
- small mass, measuring around 1-2 cm
- attached to the endocardium by a short pedicle
- characteristic multiple papillary fronds → “sea-anemone” appearance when immersed in saline solution [1-3,7-9]
Microscopically
- central avascular core: collagen, smooth muscle cells, elastic fibres
- mucopolysaccharide layer
- endothelium [2,3,7-9]
Etiology and pathogenesis
- still unclear, many hypotheses: hamartoma, true neoplasm, organizing thrombus, hyperplastic response to genetic stimuli or external factors (mechanical trauma, radiation), viral [2,7,8]
Clinical manifestations
- most are asymptomatic, diagnosed incidentally [3]
- the spectrum of signs and symptoms varies largely and it usually results from:
a. Embolisation of fragments of the tumor’s papillary fronds or tumor surface thrombi [2,3]:
- cerebrovascular: transient ischemic attack, stroke, retinal artery occlusion
- coronary: angina pectoris, myocardial infarction, sudden death
- peripheral: mesenteric ischemia, renal and limb infarction [2-4,7,8]
- pulmonary embolism
!!! right heart papillary fibroelastoma + patent foramen ovale → risk of systemic embolisation [2,3,8,10]
b. Obstruction of:
- filling and/or outflow tracts of ventricles
- coronary arteries ostiums
c. Interference with valve function:
- valve stenosis and/or insufficiency [2,3]
Treatment
- symptomatic + risk factors (left sided, high mobility, prior embolisation): surgery [2,3,7,8,11]
- asymptomatic + high mobility papillary fibroelastoma: surgery [2,3,7]
- patients not surgical candidates: anticoagulation therapy remains controversial [2,3,8,11]
- asymptomatic + small, non-mobile tumors: follow-up [2,3,7]
Prognosis
- surgical treatment: curative
- prognosis: excellent
- no cases of recurrence [2,3]