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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 53  |  Issue : 6  |  Page : 233-235

Papillary fibroelastoma of the aortic valve


Department of Cardiovascular Surgery, Mackay Memorial Hospital, Taipei, Taiwan

Date of Submission29-Mar-2020
Date of Decision13-May-2020
Date of Acceptance06-Jul-2020
Date of Web Publication19-Dec-2020

Correspondence Address:
Yu-Hern Tan
Mackay Memorial Hospital, No. 92, Sec. 2, Zhongshan N. Road, Taipei City 10449
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_26_20

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  Abstract 


Papillary fibroelastoma (PFE) is the second most common benign primary cardiac tumor. The clinical spectrum of PFE ranges widely from asymptomatic to life-threatening thromboembolic complications. We herein report a case of a 59-year-old woman presenting with an incidentally found aortic valve tumor treated with valve-sparing surgical excision. There is no guideline for the management of PFE, but surgical excision is generally suggested for left-sided PFE.

Keywords: Cardiac tumor, fibroelastoma, papillary fibroelastoma


How to cite this article:
Tan YH, Chien CY. Papillary fibroelastoma of the aortic valve. Formos J Surg 2020;53:233-5

How to cite this URL:
Tan YH, Chien CY. Papillary fibroelastoma of the aortic valve. Formos J Surg [serial online] 2020 [cited 2021 Jan 23];53:233-5. Available from: https://www.e-fjs.org/text.asp?2020/53/6/233/304019




  Introduction Top


Primary cardiac tumors are very rare, with an estimated incidence of about 0.02% in autopsy series.[1] Papillary fibroelastoma (PFE) is the second most common benign primary cardiac tumors affecting mostly the heart valves.[2] They account for <10% of all cardiac tumors. We here report a case of a 59-year-old woman presenting with an incidentally found aortic valve tumor.


  Case Report Top


A mass lesion on the aortic valve was incidentally found in a 59-year-old woman during the medical checkup. She had a medical history of dyslipidemia and left breast cancer status postmodified radical mastectomy and adjuvant chemotherapy. The transthoracic echocardiography revealed a mobile lesion at the base of the aortic valve, about 1 cm × 0.5 cm in size. The mass causes neither outflow tract obstruction nor aortic valve regurgitation. Subsequent transesophageal echocardiography [Figure 1] and computed tomography also demonstrated the presence of the lesion abutting the noncoronary cusp of the aortic valve [Figure 2]. The differential diagnosis included tumor, vegetation, or thrombus.
Figure 1: (a and b) Transesophageal echocardiography showing a mass lesion of 1 cm × 0.5 cm in size at the noncoronary cusp of the aortic valve

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Figure 2: A pedunculated mass attached to the noncoronary cusp of the aortic valve

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Due to the highly mobile nature of the tumor with the possible risk of embolization, she underwent surgery following a thorough heart team discussion. The surgery was performed under mild hypothermic cardiopulmonary bypass (32°C) through standard ascending aortic and bicaval cannulation. After aortic cross-clamp and antegrade infusion of cardioplegic solution through the aortic root, transverse aortotomy was performed. During exploration, a yellowish, firm, and pedunculated tumor was found to be attached to the noncoronary cusp of the aortic valve [Figure 3]. The tumor was successfully excised without damaging the aortic valve. The aortic cross-clamp time and cardiopulmonary bypass time were 37 and 53 min, respectively. Postoperative transesophageal echocardiography confirmed the complete excision of the tumor and the absence of aortic valve regurgitation.
Figure 3: Gross specimen of the resected tumor

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Histopathological examination of the resected tumor confirmed the diagnosis of PFE. The lesion consisted of avascular papillary fronds lined by bland-looking, flat-to-cuboid endothelium. Focal edematous change was observed in the fibroelastic core [Figure 4].
Figure 4: Microscopic appearance of the resected tumor showing avascular, branching fronds lined by the endothelial cells (H and E stain, ×40)

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The postoperative course was uneventful, and the patient was discharged 7 days after the surgery without complication. At 1-year follow-up, no recurrence of tumor or aortic regurgitation has been found.


  Discussion Top


PFE is the second most common benign primary cardiac tumor.[2] PFE most frequently arose on the aortic valve, followed by mitral valve, tricuspid valve, and pulmonary valve.[2],[3],[4] Grossly, these tumors have a characteristic frond-like appearance resembling sea anemones.[5] Most of them were within 1 cm of size. Histologically, PFEs are constituted of central avascular core surrounded by a layer of endothelium.[2],[5] This is quite different from the myxomas, which are mainly composed of stellate and fusiform myxoma cells within myxoid matrix of acid mucopolysaccharide.[6]

The definite diagnosis of PFE is based on histopathological examination. No definite symptoms are specifically suggestive of PFE. The clinical spectrum of PFE ranges widely from asymptomatic to life-threatening thromboembolic complications.[7] Most of the PFEs are asymptomatic and found incidentally at the time of echocardiography or cardiac surgery. However, case reports of neurological events, acute myocardial infarction, and pulmonary emboli related to PFEs are not infrequent.

To date, there is no guideline on the management of PFE. There is no doubt that symptomatic lesions should be removed surgically.[2] Generally, the decision of surgical excision for asymptomatic lesion is made based on the risk stratification including the size, location, mobility, potential of embolization, and surgical risks.[7],[8] Gowda et al.[2] have recommended surgical removal of asymptomatic, mobile PFE regardless of tumor size owing to increased risk of embolic events. Tamin et al.[8] have suggested surgical intervention in the left-sided PFE regardless of size, mobility, and symptoms in patients with low surgical risk.


  Conclusion Top


Although PFEs are benign in nature, they carry a risk of disastrous complications secondary to thromboembolic events, especially in the left-sided tumors. Surgical removal should be considered once diagnosis is made.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Reynen K. Frequency of primary tumors of the heart. Am J Cardiol 1996;77:107.  Back to cited text no. 1
    
2.
Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ. Cardiac papillary fibroelastoma: A comprehensive analysis of 725 cases. Am Heart J 2003;146:404-10.  Back to cited text no. 2
    
3.
Anastacio MM, Moon MR, Damiano RJ Jr., Pasque MK, Maniar HS, Lawton JS. Surgical experience with cardiac papillary fibroelastoma over a 15-year period. Ann Thorac Surg 2012;94:537-41.  Back to cited text no. 3
    
4.
Ngaage DL, Mullany CJ, Daly RC, Dearani JA, Edwards WD, Tazelaar HD, et al. Surgical treatment of cardiac papillary fibroelastoma: A single center experience with eighty-eight patients. Ann Thorac Surg 2005;80:1712-8.  Back to cited text no. 4
    
5.
Val-Bernal JF, Mayorga M, Garijo MF, Val D, Nistal JF. Cardiac papillary fibroelastoma: Retrospective clinicopathologic study of 17 tumors with resection at a single institution and literature review. Pathol Res Pract 2013;209:208-14.  Back to cited text no. 5
    
6.
Gošev I, Paić F, Durić Z, Gošev M, Ivčević S, Jakuš FB, et al. Cardiac myxoma the great imitators: Comprehensive histopathological and molecular approach. Int J Cardiol 2013;164:7-20.  Back to cited text no. 6
    
7.
Sun JP, Asher CR, Yang XS, Cheng GG, Scalia GM, Massed AG, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: A retrospective and prospective study in 162 patients. Circulation 2001;103:2687-93.  Back to cited text no. 7
    
8.
Tamin SS, Maleszewski JJ, Scott CG, Khan SK, Edwards WD, Bruce CJ, et al. Prognostic and bioepidemiologic implications of papillary fibroelastomas. J Am Coll Cardiol 2015;65:2420-9.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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