Cardiac papillary fibroelastoma: experience of an institution

Abstracts

Primary intracardiac tumors are rare, with prevalence between 0.0017% and 0.19% from non-selected autopsy studies. Approximately 75% are benign and almost half of them are myxomas. The remaining tumors are divided among rabdomyomas, lipomas and fibroelastomas. Myxomas are the most common intracardiac tumors in adult age and rabdomyomas the most common among pediatric population. Papillary fibroelastoma (PFE) is a relative rare benign heart tumor, corresponding to approximately 8% of intracardiac tumors. They most commonly manifested in cardiac valves¹. In the past, they either consisted of necropsy findings or were found in surgical procedures at random. In vivo diagnosis was sporadic². With the improvement of echocardiography techniques, PFE has been more frequently diagnosed. They are usually described as a movable, pedunculate, well-delimited mass and with predilection for valve endocardium. Therapeutic proposal, when they are pedunculate, is surgical resection, preventing cerebral, pulmonary, coronary or peripheral embolic phenomena1,3. Five cases diagnosed in our institution, in the period from August 1995 to June 2004, will be presented

Os tumores cardíacos primários do coração são raros, com uma prevalência entre 0,0017% e 0,19% dos estudos de autópsia não selecionados. Cerca de 75% são tumores benignos e quase a metade são mixomas. Os restantes se dividem entre rabdomiomas, lipomas e fibroelastomas. Os mixomas são os tumores cardíacos mais comuns na idade adulta e os rabdomiomas, os mais comuns da população pediátrica. O fibroelastoma papilífero (FEP) é um tumor benigno do coração, relativamente raro, correspondendo a aproximadamente 8% dos tumores cardíacos. São os que mais comumente acometem as valvas cardíacas¹. No passado, consistiam de achados de necropsia ou eram encontrados em procedimentos cirúrgicos ao acaso. O diagnóstico in vivo era esporádico². Com o aprimoramento das técnicas de ecocardiografia, o FEP tem sido diagnosticado com maior freqüência. São, geralmente, descritos como uma massa móvel, pedunculada, bem delimitada e com predileção pelo endocárdio valvar. A proposta terapêutica, quando pedunculados, é a ressecção cirúrgica, visando a prevenção de fenômenos embólicos cerebrais, pulmonares, coronarianos ou periféricos1,3. Serão apresentados cinco casos diagnosticados em nossa instituição, no período de agosto de 1995 a junho de 2004.

CASE REPORT

Cardiac papillary fibroelastoma. Experience of an institution

Suzelle F. de M. Oliveira; Ricardo Ribeiro Dias; Fábio Fernandes; Noedir A. G. Stolf; Charles Mady; Sérgio Almeida de Oliveira

IInstituto do Coração do Hospital das Clínicas (FMUSP) - São Paulo, SP - Brazil

^{Correspondence}

ABSTRACT

Primary intracardiac tumors are rare, with prevalence between 0.0017% and 0.19% from non-selected autopsy studies. Approximately 75% are benign and almost half of them are myxomas. The remaining tumors are divided among rabdomyomas, lipomas and fibroelastomas. Myxomas are the most common intracardiac tumors in adult age and rabdomyomas the most common among pediatric population.

Papillary fibroelastoma (PFE) is a relative rare benign heart tumor, corresponding to approximately 8% of intracardiac tumors. They most commonly manifested in cardiac valves¹. In the past, they either consisted of necropsy findings or were found in surgical procedures at random. In vivo diagnosis was sporadic². With the improvement of echocardiography techniques, PFE has been more frequently diagnosed. They are usually described as a movable, pedunculate, well-delimited mass and with predilection for valve endocardium. Therapeutic proposal, when they are pedunculate, is surgical resection, preventing cerebral, pulmonary, coronary or peripheral embolic phenomena^1,3. Five cases diagnosed in our institution, in the period from August 1995 to June 2004, will be presented.

Case Reports

Case 1 - A 27-year-old female patient, under Turner Syndrome follow-up, sent due to echocardiographic finding of tumoral, pedunculate, movable in tricuspid valve topography image. She did not show symptoms and her physical exam was normal, except for bodily marks from the syndrome itself. Echocardiogram evidenced a round echodense movable image, located at right atrium, adhered to apical portions of tricuspid valve septal leaflet, with signs of left ventricular filling obstructions. The 1.8 cm x 1.2 cm mass was submitted to surgical exeresis (fig. 1). Microscopic exam confirmed papillary fibroelastoma diagnosis.

Case 2 - Female patient, 67 years old, clinically asymptomatic, submitted to physical examination in which discreet systolic cardiac murmur was detected. Transesophageal echocardiogram showed pedunculate movable mass in aortic valve, which moved towards the aorta (fig. 2). Magnetic nuclear resonance imaging evidenced the referred mass in aortic valve leaflet (fig. 3). She was submitted to tumor exeresis. Diagnostic confirmed papillary fibroelastoma.

Case 3 - A 63-year-old female diabetic patient, with mitral commissurotomy history due to rheumatic mitral stenosis and chronic atrial fibrillation. She made use of oral anticoagulant with coumarinic and showed recurring episodes of transitory ischemic attack (TIA). Echocardiogram evidenced mitral stenosis with valve area of 1.4 cm² and anomalous echoes of 1.8 cm x 1.8 cm in tendinous chord, suggestive of vegetation. There were left atrium thrombus signs. She was sent to surgical treatment and, as a finding, a characteristic tumoral formation with a diameter of, 2 cm, which extended to the papillary muscle, was evidenced in mitral valve anterior leaflet. The exchange of mitral valve through biological prosthesis and tumor exeresis were performed. Microscopic exam confirmed papillary fibroelastoma diagnosis (fig. 4).

Case 4 - Male patient, 59 years old, with history severe mitral regurgitation due to myxomatous degeneration, made use of coumarinic in force of chronic atrial fibrillation and showed a TIA episode. He was under a follow-up due to functional class III heart failure (NYHA). He was submitted to surgical treatment, in which chord rupture was evidenced and quadrangular resection of posterior cuspid was carried out, followed by raffia and posterior annuloplasty with a bovine pericardium strip. Besides chronic valvopathy with fibrosis and myxoid material deposit, tumorgenicity compatible with papillary fibroelastoma was evidenced in microscopic exam.

Case 5 - A 49-year-old male patient, with history of rheumatic mitral lesion, under follow-up due to functional class III (NYHA). He was submitted to mitral valve exchange for biological prosthesis. Histopathological finding of material sent for study was compatible with papillary fibroelastoma, located in one of tendinous chords.

Patients evolved without immediate complications and free from reincidences in all five cases.

Discussion

Papillary fibroelastoma is a low-prevalence benign tumor, with tendency to valvar involvement^2,4,5. Concerning frequency, it corresponds to the third most common primary intracardiac tumor, preceded by myxomas and lipomas⁶. It represents less than 10% from all primary intracardiac tumors, either those studied in autopsy or after resection^1,7,8.

Approximately 90% of PFE undertakes cardiac valves, usually as a single lesion, on the atrial face of atrioventricular valves or on any one of the sides of semilunar valves^3,4. They rarely occur as multiple lesions^1,6.

Approximately 44% of PFE is found in aortic valves, followed by the undertaking of mitral valve in 35% of the cases, in 15%, in tricuspid valves and in 8%, in pulmonary ones⁹. Reports of cases on those tumors have demonstrated undertaking of all endocardial surfaces, including papillary muscles, tendinous chords, the septum or free walls from any of cardiac chambers^1,5,8,10.

The size of described PFE varied from 0.1 to 4 cm, and most of them were smaller than 1cm of diameter⁴. Their genesis remains controversial. Real neoplasias until hamartomas, organized thrombi, reactive responses to mechanic trauma, to surgical or radiotherapy damage^1,3,4,8,9 have been considered. Prevalence is unknown due to the group of non-diagnosed silent tumors⁴.

The age of patients is variable, from cases in neonates to well-advanced age patients^1,4,7. Most of it is described in adults, over 50 years of age, and there is no difference between sexes^1,4,9.

PFE is an incidental finding in most cases, although among symptomatic patients the clinical presentation is variable and dependent on location, motility and size of tumor^4,9. As most of it originates from left chambers (more than 95% of the cases), the most feared complication is the systemic embolization, especially for cerebral or coronary circulation^4,9. It is not clear whether the embolus is tumor- or platelet-origin and if systemic anticoagulation could prevent from such events^2,4,9. The most common clinical presentation described was stroke or transitory ischemic attack (TIA). Other described manifestations were: angina, myocardial infarction, sudden death, heart failure, syncope, pulmonary embolism, blindness, peripheral embolism of renal infarction⁹. In aortic valve tumor patients, sudden death and myocardial infarction were the most common manifestations. In rebuttal, stroke was the prevailing presentation⁹ in mitral valve tumors. Tumoral motility is the only independent mortality and non-fatal embolization predictor⁹.

Electrocardiographic findings are non-specific, as atrial arrhythmias may occur. Thoracic radiography may demonstrate signs of increase of cardiac chambers, pulmonary hypertension or congestion, especially if the tumor is occluding the mitral valve. Transthoracic echocardiogram is the ideal method for tumor diagnosis and characterization, as it usually demonstrates the mass with its varied proportions, motile or not, well-delimited, pedunculate or sessile, of round, oval or irregular shape². They are mostly small (99% smaller than 2.0 cm)². In a case-control study, the sensitivity and specificity of echocardiogram was 88.9% and 87.8%, respectively². Magnetic resonance imaging demonstrates the mass in valve leaflet or cardiac chamber, and the presence of enhancement with gadolinium in tumoral mass, increases the suspicion level⁹. Cardiac catheterization does not contribute for the diagnosis. In coronary angiography it is possible to visualize total occlusions of coronary arteries, as well as aneurysmatic dilatations and secondary narrowing to tumoral emboli^1,9.

PFE has characteristic appearance, resembling a sea anemone, with multiple ramifications held by a pedicle to endocardium. At histological exam, it consists of an endothelium coat, which covers a connective tissue matrix with variable amounts of collagen, smooth muscle cells and elastic fibers^2,4.

For symptomatic patients, surgical exeresis is the choice for treatment, by trying to always preserve valve tissue and its function⁷. Among asymptomatic individuals, surgical procedure is controversial, as tumoral motility is the determining factor of surgical indication, for being an independent embolization and death predictor. Surgery is curatory and there is no report on reincidences^4,9. Follow-up of asymptomatic patients who were not submitted to surgery must include anticoagulation, although its efficacy in protection against embolic phenomena is controversial². Management before a left side isolated lesion includes surgical removal, when the mass is big and/or movable or in the presence of patent ovale foramen due to the possibility of paradoxical embolism².

In the last years, PFE has progressed from an autopsy incidental finding to an in vivo diagnosed disease with potential injurious complications, which require proper diagnosis and treatment. Due to its rareness, data on the treatment and follow-up are mostly derived from accounts from patients and experiences as those described in our work.

REFERENCES

Received on 09/09/04

Accepted on 03/04/05

• 1. Pacini D, Farneti PA, Leone O, Galli R. Cardiac papillary fibroelastoma of the mitral valve chordae. Eur J Cardiothoracic Surg 1998; 13: 322-4.
• 2. Sun JP, Asher CR, Yang XS et al. Clinical and echocardiographic characteristics of papillary fibroelastomas. Circulation 2001; 103: 2687-93.
• 3. Kurup AN, Tazelaar, HD, Edwards WD et al. Iatrogenic cardiac papillary fibroelastoma: A study of 12 Cases (1990 to 2000). Hum Pathol 2002; 33: 1165-9.
• 4. Shahian DM, Labib SB, Chang G. Cardiac papillary fibroelastoma. Ann Thorac Surg 1995; 59: 538-41.
• 5. Gologorsky E, Gologorsky A. Aortic valve fibroelastomas as an incidental intraoperative transesophageal echocardiographic finding. Anesth Analg 2002; 95: 1198-9.
• 6. Tanaka H, Narisawa T, Mori T et al. Double Primary Left Ventricular and Aortic Valve Papillary Fibroelastoma. Circ J 2004; 68: 504-6.
• 7. Di Mattia DG, Assaghi A, Mangini A et al. Mitral valve repair for anterior leaflet papillary fibroelastoma: two case descriptions and a literature review. Eur J Cardiothoracic Surg 1999; 15: 103-7.
• 8. Georghiou GP, Erez E, Vidne BA, Aravot D. Tricuspid valve papillary fibroelastoma: an unusual cause of intermittent dyspnea. Eur J Cardiothoracic Surg 2003; 23: 429-31.
• 9. Gowda RM, Khan IA, Nair CK et al. Cardiac papillary fibroelastoma: A comprehensive analysis of 725 cases. Am Heart J 2003; 146: 404-10.
• 10. Gowda RM, Khan IA, Mehta NJ et al. Cardiac Papillary Fibroelastoma originating from pulmonary vein a case report. Angiology 2002; 53: 745-8.

Publication Dates

• Publication in this collection
  10 Oct 2005
• Date of issue
  Sept 2005

History

• Accepted
  04 Mar 2005
• Received
  09 Sept 2004