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Year : 2008  |  Volume : 51  |  Issue : 4  |  Page : 559-560
Papillary fibroelastoma of the heart


Department of Pathology, T.N. Medical College and B.Y.L. Nair Hospital, Dr. A.L. Nair Road Mumbai Central, Mumbai 400 008, India

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   Abstract 

Primary intra-cardiac tumors are rare. Most of them are benign. Of the benign tumors, myxomas are the most common. Others are lipoma, rhabdomyoma, hemangioma, and papillary fibroelastoma (PFE). PFE is a relatively rare benign tumor of the heart. It occurs commonly on cardiac valves and is often an incidental finding. They are most commonly discovered during autopsy, but may present with thromboembolism, which is a dreaded complication. It is important to be aware of this entity because even though it is benign it may present with life-threatening complications, which are well documented in literature. Surgery is the treatment of choice for these tumors. We present the case of a 30-year-old male in whom PFE was an incidental finding

Keywords: Cardiac tumors, papillary fibroelastoma

How to cite this article:
Ahuja JR, Amonkar GP, Deshpande J. Papillary fibroelastoma of the heart. Indian J Pathol Microbiol 2008;51:559-60

How to cite this URL:
Ahuja JR, Amonkar GP, Deshpande J. Papillary fibroelastoma of the heart. Indian J Pathol Microbiol [serial online] 2008 [cited 2020 May 29];51:559-60. Available from: http://www.ijpmonline.org/text.asp?2008/51/4/559/43762



   Introduction Top


Primary intra-cardiac tumors are rare. The incidence ranges from 0.0017-0.33% according to various studies. [1],[2],[3] Approximately 50-80% of tumors are benign. [1],[2],[3] The most common benign tumor is myxoma and the most common malignant tumor is angiosarcoma. [1],[2],[3] The other benign tumors are PFE, lipoma, rhabdomyoma, and hemangioma. [1],[2],[3] Other malignant tumors are malignant fibrous histiocytoma, rhabdomyosarcoma, and leiomyosarcoma. [1],[2] Metastasis to the heart is commonly seen from primary malignancies of the lung, breast, and cutaneous melanoma. [1] PFEs are extremely rare, benign primary cardiac tumors. We present an interesting case of PFE in a 30-year-old male, which was an incidental finding.


   Case Report Top


Our case is a 30-year-old male who had a known case of rheumatic heart disease. We received specimens of the mitral and aortic valves. Grossly the mitral valve leaflets were thickened, the commissures were fused, and the chordae tendinae were shortened. The cusps of the aortic valve were thickened and it showed a small papillary growth, which was 0.8 cm in diameter. The microscopy showed healed valvulitis in the mitral valve. The aortic valve showed neovascularization, sparse inflammatory cells, and a few small collections of histiocytes with lymphocytes-features of healed valvulitis. Sections from the papillary growth revealed small papillary projections lined by cuboidal epithelium with a central fibrovascular core [Figure 1]. A diagnosis of papillary fibroelastoma was made.


   Discussion Top


PFE is a rare tumor of the heart. [1] It accounts for less than 10% of the primary intra-cardiac tumors. [1],[2],[3] PFE is the most common benign tumor of the valves, it occurs most commonly on the valves; [1] and it occurs most commonly on the left side of the heart. [4],[5] The aortic valve is most commonly involved followed by the anterior and posterior leaflets of the mitral valve. [1] Sometimes, other valves, chordae, and papillary muscles may be involved. [1]

According to one study, the aortic valve is involved in 44% of cases, the mitral valve is involved in 15% of cases, and the pulmonary valve is involved in 8% of cases. [6] In our case, the aortic valve was involved. They occur at any age but peak at 60 years and no gender predilection is seen. [1],[7] Our case is a 30-year-old male. These tumors are most commonly seen on diseased valves like post-rheumatic valves and valves with fibrosis or calcification. In most cases, PFE is an incidental finding. In our case, PFE was an incidental finding on a diseased rheumatic valve.

The symptomatic cases present with symptoms related to the location of the tumor and since it is most commonly on the left side, the risk of presentation with thromboembolism is high. [1],[8] The most useful investigation for diagnosis is multiplanar transthoracic oesophageal echocardiography. [1],[3],[9] Macroscopically, they are 2-50 mm in greatest dimensions although the majority are less than 10 mm. [1],[2] They are generally solitary, opalescent white, and attached to the valve by a single stalk. [1],[6] The diseased valve should be carefully examined for papillary excresences. Grossly, Lambl's excrescences is a differential diagnosis but these occur on the line of closure of the valves while PFE can occur anywhere on the valve surface. [1] PFE also shows the presence of elastic fibers within the central core. Microscopically, they are papillary structures that have a superficial endothelial layer beneath which is a layer rich in proteoglycans. These papillae have a fibrovascular core. Inner layers contain fibroblasts and may occasionally show macrophages and dendritic cells. [1]

Surgery is the treatment of choice. The lesion is removed with preservation of as much valve function as possible. Recurrence has not been reported. [5],[6] Surgery in asymptomatic cases is controversial. Cases in which surgery has not been done should be followed up with anticoagulants although its efficacy against embolism is controversial. [9]

 
   References Top

1.Burke AP, Tazelaar H, Gomez-Roman JJ, Loire R, Chopra P, Tomsova M, et al. Benign tumours of pluripotent mesenchyme. In: Travis WT, Brambilla E, Muller-Hermelink HK, Harris CC, editors. Pathology and genetics of tumours of the lung, pleura and heart. World Health Organisation Classification of tumours. Lyon: IARC; 2004. p. 260-5.   Back to cited text no. 1    
2.Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T. Cardiac tumours: Diagnosis and management. Lancet Oncol 2005;6:219-28.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Meng Q, Lai H, Lima J, Tong W, Qian Y, Lai S. Echocardiographic and pathologic characteristics of primary cardiac tumors: A study of 149 cases. Int J Cardiol 2002;84:69-75.   Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Tanaka H, Narisawa T, Mori T, Masuda Y, Kishi D. Double primary left ventricular and aortic valve papillary fibroelastoma. Circ J 2004;68:504-6.   Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BG, Sacchi TJ. Cardiac papillary fibroelastoma: A comprehensive analysis of 725 cases. Am Heart J 2003;146:404-10.   Back to cited text no. 5    
6.Shahian DM, Labib SB, Chang G. Cardiac papillary fibroelastoma. Ann Thorac Surg 1995;59:538-41.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Hindupur S, Schwabe JL. Papillary Fibroelastoma of the right atrium: An atypical presentation, A case report and review of the literature. J Cardiovasc Surg (Torino) 2005;46:589-91.  Back to cited text no. 7  [PUBMED]  
8.Fox E, Brunson C, Campbell W, Aru G. Cardiac papillary fibroelastoma presents as an acute embolic stroke in a 35-year old African American male. Am J Med Sci 2006;331:91-4.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Sun JP, Asher CR, Yang XS, Cheng GG, Scallia 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. 9    

Top
Correspondence Address:
Gayathri P Amonkar
Department of Pathology, T.N. Medical College, B.Y.L. Nair Hospital, Dr. A.L. Nair Road, Mumbai Central, Mumbai 400008, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.43762

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