Papillary fibroelastoma

A papillary fibroelastoma is a rare benign primary cardiac tumour. However of the primary cardiac tumours, they are one of the commonest to occur in relation to the cardiac valves.

Epidemiology

Its estimated prevalence ranges between < 0.01 to 0.33 % 5.

Pathology

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.

Location

~ 80% occur in relation to either the aortic of 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 mediate 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.

Article Author : Dr Yuranga Weerakkody, Radiopaedia

References

* 1 Markus Kondruweit et.al, Papillary fibroelastoma of the mitral valve: appearance in 64-slice spiral computed tomography, magnetic resonance imaging, and echocardiography ; Eur Heart J (2007) doi: 10.1093/eurheartj/ehm475
* 2. Philip A. Araoz et.al, CT and MR Imaging of Primary Cardiac Malignancies, November 1999 RadioGraphics, 19, 1421-1434.
* 3. David H. O'Donnell et.al, Review : Cardiac Tumors: Optimal Cardiac MR Sequences and Spectrum of Imaging Appearances, AJR 2009; 193:377-387
* 4. Anil K. Attili1 and Felix S. Chew ;Imaging of Cardiac Masses and Myocardial Disease: Self-Assessment Module, AJR 2007; 188:S21-S25
* 5. Damodar Kumbala, “Perilous Pearl”—Papillary Fibroelastoma of Aortic Valve: A Case Report and Literature Review, ANGIOLOGY October 2008 vol. 59 no. 5 625-628

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