Papillary fibroelastoma of the heart

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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 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 <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>
+<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 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><ul>
+<li><a href="/articles/fibroelastoma-of-mitral-valve">fibroelastoma of mitral valve</a></li>
+<li><a href="/articles/fibroelastoma-of-aortic-valve">fibroelastoma of aortic valve</a></li>
+<li><a href="/articles/fibroelastoma-of-tricuspid-valve">fibroelastoma of tricuspid valve</a></li>
+</ul><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>

References changed:

4. Attili AK, Chew FS. Imaging of cardiac masses and myocardial disease: self-assessment module. AJR Am J Roentgenol. 2007;188 (6_supplement): S21-5. <a href="http://dx.doi.org/10.2214/AJR.07.0193">doi:10.2214/AJR.07.0193</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/19642248">Pubmed citation</a><span class="auto"></span>
5. Kumbala D, Sharp T, Kamalesh M. "Perilous pearl"-papillary fibroelastoma of aortic valve: a case report and literature review. Angiology. 2008;59 (5): 625-8. <a href="http://dx.doi.org/10.1177/0003319707305986">doi:10.1177/0003319707305986</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/18388078">Pubmed citation</a><span class="auto"></span>
6. Shah RA, Kalidoss L, Mohanraj A et-al. Papillary fibroelastoma of tricuspid valve presenting as pulmonary embolism. Asian Cardiovasc Thorac Ann. . <a href="http://dx.doi.org/10.1177/0218492314526601">doi:10.1177/0218492314526601</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/24604555">Pubmed citation</a><span class="auto"></span>
7. Ibrahim M, Masters RG, Hynes M et-al. Papillary fibroelastoma of the pulmonary valve. Can J Cardiol. 2006;22 (6): 509-10. <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2560554">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/16685317">Pubmed citation</a><span class="auto"></span>
8. Fonseca P, Neves P, Ribeiro J et-al. Papillary fibroelastoma of the pulmonary valve. Rev Port Cardiol. 2014;33 (1): 57-8. <a href="http://dx.doi.org/10.1016/j.repc.2013.06.016">doi:10.1016/j.repc.2013.06.016</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/24393338">Pubmed citation</a><span class="auto"></span>
9. Hakim FA, Aryal MR, Pandit A et-al. Papillary fibroelastoma of the pulmonary valve-a systematic review. Echocardiography. 2014;31 (2): 234-40. <a href="http://dx.doi.org/10.1111/echo.12388">doi:10.1111/echo.12388</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/24128270">Pubmed citation</a><span class="auto"></span>
10. van Werkum MH, Swaans MJ, van Es HW et-al. Case 190: papillary fibroelastoma of the pulmonary valve. Radiology. 2013;266 (2): 680-4. <a href="http://dx.doi.org/10.1148/radiol.12110495">doi:10.1148/radiol.12110495</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/23362097">Pubmed citation</a><span class="auto"></span>
11. Gopaldas RR, Atluri PV, Blaustein AS, Bakaeen FG, Huh J, Chu D. Papillary fibroelastoma of the aortic valve: operative approaches upon incidental discovery. Texas Heart Institute journal. 36 (2): 160-3. <a href="https://www.ncbi.nlm.nih.gov/pubmed/19436815">Pubmed</a> <span class="ref_v4"></span>
12. Fibroelastoma and Embolic Stroke. Circulation. 99 (20): 2709. <a href="https://doi.org/10.1161/01.CIR.99.20.2709">doi:10.1161/01.CIR.99.20.2709</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/10338468">Pubmed</a> <span class="ref_v4"></span>
1. Kondruweit M, Schmid M, Strecker T. Papillary fibroelastoma of the mitral valve: appearance in 64-slice spiral computed tomography, magnetic resonance imaging, and echocardiography. Eur. Heart J. 2008;29 (6): 831. <a href="http://dx.doi.org/10.1093/eurheartj/ehm475">doi:10.1093/eurheartj/ehm475</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/17959625">Pubmed citation</a><span class="auto"></span>
3. O'Donnell DH, Abbara S, Chaithiraphan V et-al. Cardiac tumors: optimal cardiac MR sequences and spectrum of imaging appearances. AJR Am J Roentgenol. 2009;193 (2): 377-87. <a href="http://dx.doi.org/10.2214/AJR.08.1895">doi:10.2214/AJR.08.1895</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/19620434">Pubmed citation</a><span class="auto"></span>
4. Attili AK, Chew FS. Imaging of cardiac masses and myocardial disease: self-assessment module. AJR Am J Roentgenol. 2007;188 (6_supplement): S21-5. <a href="http://dx.doi.org/10.2214/AJR.07.0193">doi:10.2214/AJR.07.0193</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/19642248">Pubmed citation</a><span class="auto"></span>
5. Kumbala D, Sharp T, Kamalesh M. "Perilous pearl"-papillary fibroelastoma of aortic valve: a case report and literature review. Angiology. 2008;59 (5): 625-8. <a href="http://dx.doi.org/10.1177/0003319707305986">doi:10.1177/0003319707305986</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/18388078">Pubmed citation</a><span class="auto"></span>
10. van Werkum MH, Swaans MJ, van Es HW et-al. Case 190: papillary fibroelastoma of the pulmonary valve. Radiology. 2013;266 (2): 680-4. <a href="http://dx.doi.org/10.1148/radiol.12110495">doi:10.1148/radiol.12110495</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/23362097">Pubmed citation</a><span class="auto"></span>
8. Fonseca P, Neves P, Ribeiro J et-al. Papillary fibroelastoma of the pulmonary valve. Rev Port Cardiol. 2014;33 (1): 57-8. <a href="http://dx.doi.org/10.1016/j.repc.2013.06.016">doi:10.1016/j.repc.2013.06.016</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/24393338">Pubmed citation</a><span class="auto"></span>
9. Hakim FA, Aryal MR, Pandit A et-al. Papillary fibroelastoma of the pulmonary valve-a systematic review. Echocardiography. 2014;31 (2): 234-40. <a href="http://dx.doi.org/10.1111/echo.12388">doi:10.1111/echo.12388</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/24128270">Pubmed citation</a><span class="auto"></span>
6. Shah RA, Kalidoss L, Mohanraj A et-al. Papillary fibroelastoma of tricuspid valve presenting as pulmonary embolism. Asian Cardiovasc Thorac Ann. . <a href="http://dx.doi.org/10.1177/0218492314526601">doi:10.1177/0218492314526601</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/24604555">Pubmed citation</a><span class="auto"></span>
7. Ibrahim M, Masters RG, Hynes M et-al. Papillary fibroelastoma of the pulmonary valve. Can J Cardiol. 2006;22 (6): 509-10. <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2560554">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/16685317">Pubmed citation</a><span class="auto"></span>
3. O'Donnell DH, Abbara S, Chaithiraphan V et-al. Cardiac tumors: optimal cardiac MR sequences and spectrum of imaging appearances. AJR Am J Roentgenol. 2009;193 (2): 377-87. <a href="http://dx.doi.org/10.2214/AJR.08.1895">doi:10.2214/AJR.08.1895</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/19620434">Pubmed citation</a><span class="auto"></span>
1. Kondruweit M, Schmid M, Strecker T. Papillary fibroelastoma of the mitral valve: appearance in 64-slice spiral computed tomography, magnetic resonance imaging, and echocardiography. Eur. Heart J. 2008;29 (6): 831. <a href="http://dx.doi.org/10.1093/eurheartj/ehm475">doi:10.1093/eurheartj/ehm475</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/17959625">Pubmed citation</a><span class="auto"></span>