Papillary fibroelastoma of the heart
Updates to Article Attributes
Papillary fibroelastomas are rare benign primary cardiac tumours. However, of the primary cardiac tumours, they are one of the commonest to occur in relation to the cardiac valves (may account for 75% of valvular tumours 10).
Epidemiology
Its estimated prevalence ranges between <0.01 to 0.33% 5. There may be slightly increased male predilection 10.
Pathology
Characterised by papillary growth of stromal tissue composed of a centraldensecentral dense amorphous fibrous core and an surrounding layer of loose connectivetissueconnective tissue. There is a also a layer of elastic fibres (hallmark) which is variable in distribution. This is further surrounded by a single layeroflayer of hypertrophied endothelium.
Location
~80% occur in relation to either the aortic ofor mitral valve 2 while tricuspid and pulmonary involvement is much rarer.
Radiographic features
Cardiac MRI
Typically seen as a small (~1.5 cm 5) well defined pedunculated mass in relation to a valve.
- T1: intermediate intensity 1-2
-
T2:
inter mediateintermediate to high intensity 1-2 - SSFP Cine: may be useful for showing mobility (often highly mobile)
Complications
- systemic embolisation of tumour or thrombotic components 5
Treatment
Surgical resection is the treatment of choice.
-<p><strong>Papillary fibroelastomas</strong> are rare <a href="/articles/primary-benign-cardiac-tumours">benign primary cardiac tumours</a>. However, of the primary cardiac tumours, they are one of the commonest to occur in relation to the cardiac valves (may account for 75% of valvular tumours <sup>10</sup>). </p><h4>Epidemiology</h4><p>Its estimated prevalence ranges between <0.01 to 0.33% <sup>5</sup>. There may be slightly increased male predilection <sup>10</sup>.</p><h4>Pathology</h4><p>Characterised by papillary growth of stromal tissue composed of a centraldense amorphous fibrous core and an surrounding layer of loose connectivetissue. There is a also a layer of elastic fibres (hallmark) which is variable in distribution. This is further surrounded by a single layerof hypertrophied endothelium. </p><h5>Location</h5><p>~80% occur in relation to either the aortic of mitral valve <sup>2</sup> while tricuspid and pulmonary involvement is much rarer.</p><h4>Radiographic features</h4><h5>Cardiac MRI </h5><p>Typically seen as a small (~1.5 cm <sup>5</sup>) well defined pedunculated mass in relation to a valve.</p><ul>- +<p><strong>Papillary fibroelastomas</strong> are rare <a href="/articles/primary-benign-cardiac-tumours">benign primary cardiac tumours</a>. However, of the primary cardiac tumours, they are one of the commonest to occur in relation to the cardiac valves (may account for 75% of valvular tumours <sup>10</sup>). </p><h4>Epidemiology</h4><p>Its estimated prevalence ranges between <0.01 to 0.33% <sup>5</sup>. There may be slightly increased male predilection <sup>10</sup>.</p><h4>Pathology</h4><p>Characterised by papillary growth of stromal tissue composed of a central dense amorphous fibrous core and an surrounding layer of loose connective tissue. There is a also a layer of elastic fibres (hallmark) which is variable in distribution. This is further surrounded by a single layer of hypertrophied endothelium. </p><h5>Location</h5><p>~80% occur in relation to either the aortic or mitral valve <sup>2</sup> while tricuspid and pulmonary involvement is much rarer.</p><h4>Radiographic features</h4><h5>Cardiac MRI </h5><p>Typically seen as a small (~1.5 cm <sup>5</sup>) well defined pedunculated mass in relation to a valve.</p><ul>
-<strong>T2:</strong> inter mediate to high intensity <sup>1-2</sup>- +<strong>T2:</strong> intermediate to high intensity <sup>1-2</sup>
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