Papillary fibroelastoma of the heart

Changed by Vincent Tatco, 18 Jan 2017

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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 a papillary growth of stromal tissue composed of a central dense amorphous fibrous core and an surrounding layer of loose connective tissue. There is also a layer of elastic fibres (hallmark) which is variable in distribution. This is further surrounded by a single layer of hypertrophied endothelium. 

Location

~80% occur in relation to either the aortic or 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: intermediate 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 of the tumour 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 &lt;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 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>
  • +<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 &lt;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 a papillary growth of stromal tissue composed of a central dense amorphous fibrous core and surrounding layer of loose connective tissue. There is 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>

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