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Pathology – Research and Practice 209 (2013) 208– 214

Contents lists available at SciVerse ScienceDirect

Pathology – Research and Practice

jo u r n al hom epa ge: www.elsev ier .com/ locate /prp

riginal article

ardiac papillary fibroelastoma: Retrospective clinicopathologic studyf 17 tumors with resection at a single institution and literature review

osé Fernando Val-Bernala,∗, Marta Mayorgaa, María Francisca Garijoa,aniel Vala, Juan Francisco Nistalb

Anatomical Pathology Department, Marqués de Valdecilla University Hospital, Medical Faculty, University of Cantabria and IFIMAV, Santander, SpainCardiovascular Surgery Service, Marqués de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain

r t i c l e i n f o

rticle history:eceived 16 October 2012eceived in revised form 15 January 2013ccepted 4 February 2013

eywords:eartardiac neoplasmsapillary fibroelastomammunohistochemistry

a b s t r a c t

Cardiac papillary fibroelastomas (PFEs), which are mainly found in the valves, are rare benign tumorsthat can cause embolism. Single-center surgical experience in the treatment of this tumor is uncommon.

All patients surgically treated for this neoplasm at our institution from January 1995 to October 2012(15 patients with 17 lesions) were queried for clinical and pathologic characteristics, and the literaturewas reviewed.

The mean age of detection was 55.8 ± 11.48 years. Twenty percent of the patients were male. Thetumor was an incidental finding in 60%. Symptoms directly related to PFEs occurred in 40% of patients.The most common clinical presentation in symptomatic cases was embolism (40%), mostly transientischemic attack or stroke. Cardiac valves were predominantly involved (76.5%); the most commonlyvalve affected was the aortic valve (29.4%), followed by the mitral valve (17.6%). Concurrent valvulardisease was observed in 41.7% of patients. The mean size of tumors was 11.4 ± 7.9 mm (range 2–25 mm).In 86.7% of the patients, the tumor was solitary. The mitral valve was the most common origin of tumor

systemic embolism. Fronds core could be the end stage of hypermature elastic fibers, which grows inapposition to young fibers. Simple surgical excision or valvular reconstruction was accomplished in mostpatients (58.3%).

PFE is usually small, appears singly, and can cause potentially serious complications. Symptomaticand left side tumors should undergo surgical excision with valve-spare surgery when possible. Surgicalremoval of PFE is safe, efficacious, and definitive.

ntroduction

Primary cardiac tumors have a low incidence and representnly 0.4% [20] to 0.8% [21] of all open heart operations. Papillarybroelastomas (PFEs) are benign avascular endocardial papillo-as predominantly affecting cardiac valves. These tumors are the

econd most frequent benign cardiac neoplasms, after myxomas,ccounting for about 15% of all primary cardiac neoplasms in openeart-operated adults [4,20]. As most patients are asymptomatic,his condition was rarely diagnosed before autopsy in the past.owever, since the introduction of echocardiography, this neo-

lasm has been more commonly and accurately reported.

Although PFEs are rare, often asymptomatic, and histopatholog-cally benign tumors, they may cause serious complications, such as

∗ Corresponding author at: Facultad de Medicina, Universidad de Cantabria, Avda.ardenal Herrera Oria s/n, E-39011 Santander, Spain. Tel.: +34 942202520x73232;ax: +34 942203492.

E-mail addresses: apavbj@humv.es, valbernal@gmail.com (J.F. Val-Bernal).

344-0338/$ – see front matter © 2013 Elsevier GmbH. All rights reserved.ttp://dx.doi.org/10.1016/j.prp.2013.02.001

© 2013 Elsevier GmbH. All rights reserved.

stroke, angina, myocardial infarction, heart failure, syncope, blind-ness, or sudden death.

Single-center experiences regarding this tumor series –although informative – often contain incomplete data. The purposeof this study was to evaluate our experience gained with this neo-plasm at our institution over the past 17 years and to review theliterature.

Methods

All patients having undergone surgery due to cardiac PFE fromJanuary 1995 to October 2012 were included in this study. Follow-ing the policy of our institution, all the materials removed, includingcomplete native heart valves, were submitted to histopathologicstudy. The study focused on patient age at surgical intervention,gender, location and size of the neoplasm, clinical presentation,

including embolic events, surgical treatment, pathologic study,follow-up, and outcome.

The imaging modality predominantly used was transtho-racic and/or transesophageal echocardiography. Other imaging

J.F. Val-Bernal et al. / Pathology – Research and Practice 209 (2013) 208– 214 209

Table 1Cardiac papillary fibroelastoma: clinicopathologic data.

Case Age (y) Sex Location Size (mm) Clinical presentation Surgical treatment Follow-up (y),outcome

1 62 F Aortic valve 3 Mitral and aortic valve disease,congestive heart failure/incidentaltumor at surgery

Aortic and mitral valvereplacement

17, alive

2 75 F Mitral valve 5 Stroke Mitral valve replacement 14, DDAR3 50 M Pulmonary

valve3 Dilated cardiomyopathy/incidental

tumor at Pathology DepartmentCardiac transplantation 16, alive

4 32 F Tricuspid valve 20 Diastolic cardiac murmur, dyspneaNYHA class II

E + tricuspid valve repair 13, alive

5 56 F Left atrium 16 Stroke SE 9, alive6 59 F Pulmonary

valve15 Hypertensive heart disease/incidental

tumor at echocardiographyE + pulmonary valve repair 8, alive

7 47 F Right ventricle 2 Incidental finding in biopsy of cardiactransplantation

No treatment 8, alive

8 54 F Aortic valve 18 Angina, sudden cardiac arrest SE 6, alive9 60 F Left ventricle 23 and 6 Stroke, Turner syndrome, hypertension SE 5, alive

10 63 M Mitral valve 8 Atrial fibrillation, rheumatic valvedisease/incidental tumor atechocardiography

Mitral valve replacement 5, alive

11 40 F Mitral valve 7 Stroke E + mitral valve repair 4, alive12 73 F Aortic valve 5 Bicuspid valve stenosis/incidental

tumor at surgeryAortic valve replacement 3, alive

13 60 M Aortic valve 3 Atrial fibrillation ascending aortaaneurysm/incidental tumor at surgery

Aortic valve replacement 3, alive

14 60 F Aortic andpulmonaryvalves

15 and 25 Paroxysmal atrial fibrillation/incidentaltumors at echocardiography

SE 1, alive

15 46 F Tricuspid valve 20 Steinert’s myotonic dystrophy, atrialflutter/incidental tumor atechocardiography

E + tricuspid valve repair 0.1, alive

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patients had more than a tumor.In five (33.3%) patients, the tumors were incidental (in three

patients the lesions were found at surgery and in two at the time

DAR, death due to another reason; E, excision; SE, simple (shave) excision.

odalities utilized were computed tomography of the chest andardiac magnetic resonance.

All tissues were fixed with buffered 10% formalin and embed-ed in paraffin. For diagnostic study, sections were stained withematoxylin and eosin, Masson trichrome, Verhoeff’s elastic stain,nd Alcian blue, pH 2.5. In addition, immunohistochemical stain-ng for vimentin (Dako FLEX Ready-to-use, Glostrup, Denmark, V9),ollagen type IV (Dako, CIV, 1:25), calretinin (Dako FLEX Ready-to-se, DAK-Calret 1), thrombomodulin (Dako, MO617, 1:50), S100rotein (Dako FLEX Ready-to-use, polyclonal), c-kit (CD117, Dako,olyclonal, 1:200), smooth muscle actin (Dako FLEX Ready-to-use,A4), CD31 (Dako FLEX Ready-to-use, JC70A), CD34 (Dako FLEXeady-to-use, QBEnd 10), fibronectin (Dako, polyclonal, 1:3000),nd human amyloid P component (Dako, polyclonal, 1:1000) waserformed in nine cases.

We have previously reported two patients from this series (cases and 14; Table 1) as case reports [24,25].

This study, which includes 15 patients with PFE, compares thebtained results with other single center studies of altogether 315atients. We considered only those surgical series that comprised5 patients. Regarding the symptomatology of patients with PFE,e classified them into two groups: (a) those in whom the tumorsere incidental, including all the patients who presented with

ymptoms due to a concurrent cardiac disease and the tumor wasetected by echocardiography, plus those in which the tumor wasound at surgery or at the time of pathologic examination and (b)hose in whom the symptoms were directly related to PFEs.

In published series, the results were obtained by calculating theeighted average.

esults

All 17 tumors from the 15 patients included in this report hadistopathologic confirmation of PFE by complete excision.

Three (20%) patients were male and 12 (80%) were female. Themean patient age was 55.8 ± 11.48 years (range 32–75).

The size of the tumor varied from 2 mm to 25 mm (mean11.4 ± 7.9 mm). The valvular surface (n = 13) was the predominantlocation of the tumor, comprising 76.5% of the total tumors and 80%of the patients. 29.4% of the tumors were located in the aortic valve,followed by the mitral valve in 17.6%, the pulmonary valve in 17.6%,and the tricuspid valve in 11.8%. The left ventricle wall was the pre-dominant non-valvular site of tumor origin (11.8%). The other sitesinvolved were the left atrium (5.9%) and the right ventricle (5.9%).Eleven (64.7%) of the tumors arose in the left heart. Two (13.3%)

Fig. 1. Papillary fibroelastoma of the pulmonary valve. Photograph of the lesionin situ obtained at the operating room. The tumor has a characteristic flower-likeappearance.

210 J.F. Val-Bernal et al. / Pathology – Research and Practice 209 (2013) 208– 214

Fig. 2. Microscopic appearance of the papillary fibroelastoma. (A) Panoramic view of the lesion showing avascular, branching fronds lined by endothelial cells (from case6 undedm aride (a stic sta

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bwrds

, H&E, original magnification, 16×). (B) The fronds consist of a fibrous core surroagnification, 100×). (C) The loose connective tissue is rich in acid mucopolysacch

re present in the core and also surround this structure (from case 6, Verhoeff’s ela

f pathologic examination). In four (26.7%) patients, symptomsccurred due to another primary heart disease, and the tumor wasn echocardiographic finding. Six (40%) patients presented withymptoms that could be directly related to a cardiac mass, andhe echocardiography confirmed the tumor. Therefore, in 60% ofatients, the tumor was an incidental finding (Table 1).

Stroke was the clinical presentation in 40% of the cases locatedn the left side of the heart. In these cases, the mitral valve was theost common origin of tumor embolism (50%).Of 12 patients with valvular PFEs, 5 (41.7%) had associated

hronic valvular disease, including fibrosis and/or calcification.Twelve valvular operations were undertaken in 11 patients

ecause of the tumor. Simple (shave) excision of the valvular tumor

as performed in 3 valves (25%). Excision with valve repair was

equired in 4 valves (33.3%) and valve replacement for concurrentegenerative valve disease in 5 valves (41.7%). Thus, valve-spareurgery was performed in 58.3%. The mean postoperative follow-up

by loose connective tissue and an endothelial lining (from case 4, H&E, originalfrom case 8, Alcian blue, original magnification, 100×). (D) Abundant elastin fibersin, original magnification, 25×).

was 7.5 years (range 1 month to 17 years). No surgical mortal-ity was observed. One patient died due to causes unrelated tothe tumor 14 years after surgery. There was no tumor recurrence(Table 1).

Grossly, PFEs showed multiple, delicate, smooth, white papil-lary fronds attached to the endocardium by a short pedicle. Thetumors had a characteristic sea anemone-like when placed in wateror saline or a flower-like appearance in situ (Fig. 1). Microscopically,the papillary fronds were narrow, slender, and branching (Fig. 2A).These fronds and the entire tumor were covered by endothelium,often prominent (Fig. 2B), which surrounded a layer of myxoidmatrix rich in acid mucopolysaccharide, positive for Alcian blue(Fig. 2C), and an inner avascular, eosinophilic, paucicellular, con-

nective tissue core. The peripheral rim and the core containedcoarse and fragmented elastic fibers (Fig. 2D). These fibers weresparse or absent in the distal portions of the papillae. Surfaceendothelial cells expressed vimentin, CD31 (Fig. 3A), CD34 (Fig. 3B),

J.F. Val-Bernal et al. / Pathology – Research and Practice 209 (2013) 208– 214 211

Fig. 3. Immunohistochemistry of the papillary fibroelastoma. The endothelial lining of the fronds is positive for CD31 (A, from case 10) and CD34 (B, from case 6). The frondss e surc case

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how a striking multilayered linear staining pattern for collagen type IV beneath thore periphery in a multilayered fashion (D, anti-human amyloid P component, from

nd thrombomodulin. Most stroma cells were positive for vimentin.ccasional spindle and stellate cells within the stroma were posi-

ive for smooth muscle actin and CD34. The tumor showed a strikingultilayered linear staining pattern for collagen type IV (Fig. 3C)

nd fibronectin beneath the endothelial surface. The periphery ofhe dense core was surrounded by concentrically oriented fibersositive for the human amyloid P component (Fig. 3D). Staining foralretinin, S100, and CD117 was negative.

eview of single-center surgical series and discussion

PFE is the second most common cardiac neoplasm after myxoma4,20,21] and the most frequent valve tumor [23]. In 2003, Gowdat al. [7] published a very informative article with a comprehen-

ive analysis of 725 cases reported in the literature using MEDLINEatabase. However, a review of a single-center surgical experienceeries with PFE [2–4,12,15,16,20,22] could be informative for theurgical pathologist. Thus, this review comprises a data collection

face (C, from case 4). The microfibril component of the elastic fibers surrounds the 4). (A–D) Original magnification, 100×.

of a total of 315 patients from a single-center experience series(Table 2).

PFE is most common in adults and is particularly frequentbetween the 4th and 8th decades of life. The mean age of detec-tion was 59.9 years, and 53.3% of the patients were male. However,some surgical series, including our own report, showed a predom-inance of women [2,4,12,16,22], perhaps reflecting referral biasrather than true incidence.

Clinical manifestations of cardiac PFEs are the result of embolicepisodes, mechanical obstruction, or valvular dysfunction.

The frequency of incidental tumors varied from 37.5% to 85.7%(mean 68.5%). These incidental tumors may be found at the timeof echocardiography (performed for a concurrent cardiac disease),cardiac surgery, or pathologic examination. Tumors <0.2 cm were

not visible by echocardiography [22]. Symptoms directly related toPFEs occurred in 31.5% of patients. The most common clinical pre-sentation was embolism (25.7%), mostly transient ischemic attackor stroke. Thus, the mean of cerebral embolism in the series was

212 J.F. Val-Bernal et al. / Pathology – Research and Practice 209 (2013) 208– 214

Table 2Reported series of surgical treatment of papillary fibroelastoma: single-center experience series.

Reference/periodof study (y)

Number ofadult patients/study period

Meanage (y)

Sex Mean size(mm)/singlelesion (%)

Site Presentation Management Follow-up time(mean)/outcome

[22]/16y 162/16 years 60 46.1% males 9/91.4 44.5% AV 84.8%incidental

Not reported 6.2 years/39patients DDAR

36.4% MV 15.2%symptoms dueto PFE

11.8% LV3.6% RV1.8% PV1.8% LA

[15]/17y 88/17 years 62 71% males Notreported/93

52% AV 47.0%incidental

83% S.Excis 3 years/Surgicalmortality 2.1%

18% LV 53.0%symptoms dueto PFE

9% VR

16% MV 6% VP6% TV 2% Other2% PV7% multiple

[4]/9.2y 10/9.2 years 58.4 40.0% males Not reported 40% AV 60.0%incidental

20% S.Excis 30.2 months/alive

30% MV 40.0%symptoms dueto PFE

60% VR

10% TV 20% VP20% RV

[16]/8.8y 5/8.8 years 53 40.0% males 19/100 20% AV 80.0%incidental

40% S.Excis Not reported/alive

60% MV 20.0%symptoms dueto PFE

20% VR

20% TV 40% VP

[3]/15y 8/15 years 52.3 62.5% males 14/87.5 44.4% AV 37.5%incidental

11.1% S.Excis Not reported/alive

44.4% MV 62.5%symptoms dueto PFE

44.4% VR

11.1% TV 44.4% VP

[20]/41y 12/41 years 53.5 66.7% males 11.3/83.3 66.7% AV 66.7%incidental

Not reported Not reported

8.3% MV 33.3%symptoms dueto PFE

8.3% AV + MV8.3% TV8.3% LV

[12]/21y 7/21 years 52.6 14% males 12.1/57.2 42.8% AV 85.7%incidental

66.7% VR Not reported

28.6% TV 14.3%symptoms dueto PFE

33.3% VP

14.3% AV + MV14.3% RA

[2]/15y 23/15 years 61.6 43% males Notreported/83

44.8% AV 43.4%incidental

37.9% S.Excis 37 months/2 casesDDAR severalmonths and 7 yearsafter surgery

31.0% MV 56.5%symptoms dueto PFE

27.6% VR

6.9% TV 0.0% VP10.3% PV 34.5% other3.4% RA3.4% LV

AV, aortic valve; DDAR, death due to another reason; LA, left atrium; LV, left ventricle; MV, mitral valve; PFE, papillary fibroelastoma; PV, pulmonary valve; RA, right atrium;RV, right ventricle; S.Excis, simple (shave) excision; TV, tricuspid valve; VP, excision with valve replacement; VR, excision with valve repair.

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2.6% (range 0–62.5%). Emboli are thought to arise either from theragile papillary fronds of the PFEs or from the thrombi that can beormed on the surface of the tumors.

PFEs often originated from the valvular endocardium. The fre-uency varied from 77% [15] to 100% [3,16] with a mean of 83%. Theortic valve (48.1%), mitral valve (28%), tricuspid valve (4.8%), andulmonary valve (2.3%) were involved in that order. Non-valvularrigin was observed in 17% of cases. In our series, location in pul-onary valve outnumbered the location in tricuspid valve. This factas also observed by Anastacio et al. [2]. The left ventricle wall was

he predominant non-valvular site of tumor origin (mean 11.6%;ange 0–18.0%).

The mitral valve was the most common origin of tumor systemicmbolism (47%) followed by aortic valve (33%). Thus, we confirmedhat mitral valve was the most common origin of embolism. Con-urrent valvular heart disease was observed in 44.2% of patientsrange 0–69.5%) [12,15,16,22].

PFEs were most commonly located on the left side of the heart.he frequency varied from 94.5 [22] to 57.1 [12], with a mean of9.8%.

The mean size of tumors was 10 mm in their greatest diameterrange 9–19 mm), but giant tumors have been reported [6]. In 89.8%f the patients, the tumor was solitary (range 57.2–100%).

Microscopically, each tumor frond core consisted of a centralbroelastic stroma surrounded by myxoid ground substance and

ined by endothelial cells. The fronds show a characteristic stain-ng with Verhoeff’s method, amyloid P component, collagen typeV, and fibronectin. The anti-human P component antibody specifi-ally marks the peripheral mantle of microfibrils of elastic fibersn the skin and the blood vessels of adults [5]. Thus, the staineveals the thinner elastic fibers located in the periphery of theore. However, this staining is not observed in the central partf the core because it is mostly composed of amorphous elastin.he core could be the advanced stage of hypermature elastic fibers,nd it could grow in apposition with young fibers. To our knowl-dge, this report is the first to use an antibody to amyloid Pomponent in PFE. The staining patterns of collagen type IV andbronectin are indicative of an excessive production of basementembrane material by endothelial cells and suggest active prolif-

rative participation of these lining cells in the formation of PFE18].

The differential diagnosis includes vegetation [10], thrombus11], Lambl’s excrescence [14], myxoma [1,8], or a calcified massn the cardiac valve (calcified amorphous tumor) [17]. Microscop-cal evaluation of these lesions and clinicopathologic correlation

ill help to reach the correct diagnosis.PFE is considered a reactive or hamartomatous entity [13]. The

rocess is generally associated with persistent turbulent blood flown the endocardium, with resulting hyperplasia due to endothelialell impairment [19]. Kurup et al. [9] reported a series of 12 casesf iatrogenic origin, including cases associated with previous car-iac surgery or thoracic irradiation. Iatrogenic PFEs are frequentlyultiple and often involve non-valvular endocardial surfaces. Case

even of our series (Table 1) was an incidental finding in a cardiaciopsy after heart transplantation in a patient subjected to iterativeardiac biopsies.

Tumor surgical management requires extracorporeal circula-ion. Care should be taken to avoid fragmentation of the tumorissue as it carries a very high risk of embolic complications. Sim-le shave excision of the tumor, without an inclusive margin oformal tissue, is usually the adequate treatment. This was the treat-ent in 61.4% of the cases (range 11.1–83%). If there is resultant

alve defect, it should be repaired. Valve repair was performedn 20.9% of the cases (range 9–66.7%). However, as the tumor isot infrequently associated with other cardiac conditions, excisionnd valve replacement constitute the elective treatment in some

[

and Practice 209 (2013) 208– 214 213

cases. This was the surgical treatment in 10.7% of the cases (range6–44.4%).

The mean follow-up for all series was 4.5 years (ranging from2.5 to 6.2 years).

There were five documented deaths, but only one was surgicalmortality in a patient with concomitant lung resection for bronchi-olitis obliterans [15].

All symptomatic PFEs should be removed unless there areconvincing contraindications, in which case anticoagulation is apermissible, but insecure alternative. Asymptomatic lesions on theleft side of the heart should be excised because of their poten-tially serious consequences, whereas those arising on the right sideof the heart can be observed with serial echocardiography. Surgi-cal removal of PFE is safe, simple, effective, and permanent [19].Recurrence of PFE after surgical excision has been reported, but itis exceptional [2]. The only case reported could be a metachronouslesion.

The long-term prognosis of these patients appears to be excel-lent.

Sources of funding

There was no external funding for this project.

Conflicts of interest

None declared.

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