Papillary fibroelastoma of the heart
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).
Its estimated prevalence ranges between <0.01 to 0.33% 5. There may be slightly increased male predilection 10.
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.
~80% occur in relation to either the aortic or mitral valve 2 while tricuspid and pulmonary involvement is much rarer.
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)
- systemic embolisation of tumour or thrombotic components 5
Surgical resection of the tumour is the treatment of choice.
- 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. doi:10.1093/eurheartj/ehm475 - Pubmed citation
- 2. Araoz PA, Eklund HE, Welch TJ et-al. CT and MR imaging of primary cardiac malignancies. Radiographics. 1999;19 (6): 1421-34. Radiographics (full text) - Pubmed citation
- 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. doi:10.2214/AJR.08.1895 - Pubmed citation
- 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. doi:10.2214/AJR.07.0193 - Pubmed citation
- 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. doi:10.1177/0003319707305986 - Pubmed citation
- 6. Shah RA, Kalidoss L, Mohanraj A et-al. Papillary fibroelastoma of tricuspid valve presenting as pulmonary embolism. Asian Cardiovasc Thorac Ann. . doi:10.1177/0218492314526601 - Pubmed citation
- 7. Ibrahim M, Masters RG, Hynes M et-al. Papillary fibroelastoma of the pulmonary valve. Can J Cardiol. 2006;22 (6): 509-10. Free text at pubmed - Pubmed citation
- 8. Fonseca P, Neves P, Ribeiro J et-al. Papillary fibroelastoma of the pulmonary valve. Rev Port Cardiol. 2014;33 (1): 57-8. doi:10.1016/j.repc.2013.06.016 - Pubmed citation
- 9. Hakim FA, Aryal MR, Pandit A et-al. Papillary fibroelastoma of the pulmonary valve-a systematic review. Echocardiography. 2014;31 (2): 234-40. doi:10.1111/echo.12388 - Pubmed citation
- 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. doi:10.1148/radiol.12110495 - Pubmed citation