Grolla et al. Journal of Cardiothoracic Surgery (2017) 12:33
DOI 10.1186/s13019-017-0592-6

CASE REPORT

Open Access

Papillary fibroelastoma, unusual cause of
stroke in a young man: a case report
Elisabetta Grolla1, Michele Dalla Vestra2, Giampaolo Zoffoli3, Riccardo D’Ascoli3, Adriana Critelli4, Rocco Quatrale4,
Domenico Mangino3* and Fausto Rigo1

Abstract
Background: Papillary fibroelastoma is the third most common primary benign tumor with an incidence of up to
0.33% in autopsy series; it accounts for approximately 75% of all cardiac valvular tumors.
Case presentation: We describe a rare case of a 28-Year-old man that while playing football, had a sudden onset
of neurological deficit: aphasia, right hemiparesis and right facial numbness. Transthoracic echocardiography (TTE)
showed a 10x10 mm mass attached to the anterior mitral valve leaflet. The patient was treated surgically for the
prevention of further embolic complications. Histologic examination of the resected mass revealed a papillary
fibroelastoma. It is the third most frequent primary cardiac tumor, after myxoma and fibroma, and the most
common primary tumor of heart valves. Despite the benign nature of this tumor, it carries very high risk of embolic
complications. The successful complete resection of the papillary fibroelastoma is curative and the long-term
postoperative prognosis is excellent.
Conclusions: Differential diagnosis of cardiac masses requires clinical informations, laboratory tests, blood cultures
and appropriate use of imaging modalities. Papillary fibroelastoma is a potential cause of embolic stroke in the
young. The prompt surgical excision of papillary fibroelastoma is curative and the long-term postoperative
prognosis is excellent.
Keywords: Papillary fibroelastoma, Cardiac tumors, Cardioembolic stroke, Cerebrovascular disease, Case report

Background
Papillary fibroelastoma is the third most common primary cardiac benign tumor with an incidence of up to
0.33% in autopsy series; it accounts for approximately
75% of all cardiac valvular tumors and affects men and
women equally with a mean age of 60 years at diagnosis.
Despite the benign nature of this tumor, it carries very
high risk of embolic complications. Here we described a
case of stroke in a 28-year-old man due to cerebral
embolization originated from a cardiac papillary
fibroelastoma.
Case presentation
A 28-years-old man was admitted to the Emergency
Department of our Hospital for the sudden onset of
aphasia, right facial numbness and right hemiparesis,
* Correspondence: Domenico.Mangino@ulss12.ve.it
3
Department of Cardiac Surgery, Ospedale dell’Angelo, Venezia-Mestre, Italy
Full list of author information is available at the end of the article

while playing football. He was not an athlete and never
performed a medical examination for sports fitness.
Nothing of significant emerged in the past medical history. Physical examination confirmed the neurological
deficit, blood pressure was 120/80, heart rate 90 bpm,
no carotid bruit detected. Blood tests (blood count, kidney and liver function, coagulation and electrolytes)
were normal. Electrocardiography (ECG) showed normal
sinus rhythm. Computer Tomography (CT) of the brain,
performed in urgency, was normal. A Magnetic Resonance (MRI) of the brain showed areas of recent ischemia
in the left cerebral hemisphere with more extensive involvement of the putamen, globus pallidus and temporal
and parietal cortical-subcortical posterior lobes (Fig. 1
panel a and b). Smaller areas of similar meaning were
detectable always on the left, adjacent parietal-occipital
sulcus and on the right in the frontal and cerebellar
regions. Some small areas compatible with vascular
outcomes were found in the cerebellum. Magnetic
Resonance Angiography (MRA) showed thrombosis of

© The Author(s). 2017 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0
International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and
reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to
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(http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

Grolla et al. Journal of Cardiothoracic Surgery (2017) 12:33

Fig. 1 Brain Magnetic Resonance shows areas of recent ischemia in
the left cerebral hemisphere with more extensive involvement of the
putamen, globus pallidus and temporal and parietal cortical-subcortical
posterior lobes

the left middle cerebral artery (Fig. 2). The patient was
hospitalized in Stroke Unit and in accordance with the
guidelines was treated with thrombolysis with r-TPA
(0.9 mg/kg). Mechanical thrombectomy was not considered as the first choice of treatment since the patient did
not have contraindications to thrombolysis; given the
good clinical response to thrombolytic treatment,

Fig. 2 Epiaortic Magnetic Resonance Angiography shows an
occlusion at the origin of the left middle cerebral artery

Page 2 of 5

mechanical thrombectomy was not considered as an
adjuntive treatment either.
Transthoracic echocardiography (TTE) showed a 10 ×
10 mm mass attached to the anterior mitral valve leaflet,
fluttering into the cardiac chambers. (Fig. 3 panel a and b).
There were no evidence of valvular stenosis, regurgitation,
or left ventricular outflow tract obstruction and left ventricular function was normal. A transesophageal echocardiography (TEE) was also performed: it confirmed the
presence of a hyper echogenic nodular and mobile mass,
attached to the atrial side of A3 scallop of the anterior
mitral leaflet; it also allowed to rule out other causes of embolism such as patent foramen oval or cardiac thrombosis
(Fig. 4). A cardiac MRI confirmed the presence of the
lesion, with a maximum size equal to about 1 cm (Fig. 5).
ECG monitoring during hospitalization in Stroke Unit and
the Holter ECG revealed no arrhythmias.
The decision was to perform the excision of the mass.
We considered the possibility to remove it in the cardiac
catheterization laboratory but the mass was apparently
too big and indented to be removed with out risk of
embolization. To avoid it we decided to perform an
open heart surgical excision. The operation was performed under normothermic cardiopulmonary bypass

Fig. 3 Transthoracic echocardiogram. The long axis (panel a) and
short axis views (panel b) show the hyperechogenous mass (white
arrow) attached to atrial side of A3 scallop of the anterior
mitral leaflet

Grolla et al. Journal of Cardiothoracic Surgery (2017) 12:33

Fig. 4 Transesophageal echocardiogram. The intercommissural view
(76°) shows a mobile nodular mass, measuring 1 × 1 cm, on the
anterior leaflet of the mitral valve

Fig. 5 The Cardiac MRI shows a round mass adhering to the mitral valve

Page 3 of 5

using ascending aortic and bicaval cannulation. After
cardiac arrest with antegrade cardioplegia, left atrium
was opened A 1-cm, gelatinous and solid looking mass
was found attached to the anterior leaflet of mitral valve
near the posteromedial mitral commisure. It was
resected with its stalk (Fig. 6). Post-operative TEE confirmed normal valvular functions and absence of residual
left atrial mass. The histological examination of the
tissue revealed a papillary fibroelastoma. The early postoperative period was uncomplicated, and the patient was
discharged on postoperative day-10. Actually remains a
permanent nuanced right hemisyndrome. A 1-month
postoperative echocardiography control showed perfect
valve function with no residual mitral regurgitation or
stenosis.

Discussion
Here we described a rare and very special case of stroke
in a 28-year-old man due to cerebral embolization originated from a cardiac papillary fibroelastoma. Stroke in
the young, with no obvious risk factors, usually requires
extensive evaluation: it is important to look for secondary cause of cerebral ischemia, searching carefully
especially diseases with potential cardioembolic.
Papillary fibroelastoma is the third most common primary benign tumor with an incidence of up to 0.33% in
autopsy series; it accounts for approximately 75% of all
cardiac valvular tumors and affects men and women
equally with a mean age of 60 years at diagnosis.
According to epidemiological data described cases rarely
involve young patients [1, 2]. Althought papillary fibroelastomas are histologically benign neoplasms, they may
result in life-threatening complications if valve obstruction or systemic embolization occurs, as described in
our patient.

Fig. 6 In the intraoperative view the lesion appears as a white round
mass, without a peduncle. The gross specimen of the mass
demonstrated gelatinous appearance and smooth surface, with many
white papillary fragments with frond-like projections, typical of
papillary fibroelastoma

Grolla et al. Journal of Cardiothoracic Surgery (2017) 12:33

Most patients are asymptomatic, but some patients
may experience cerebral embolic symptoms, such as
stroke or transient ischemic attack or angina, acute
coronary syndrome, myocardial infarction or death from
coronary ostial obstruction [3–25]: transient ischemic
attack/cerebrovascular accident is considered by far the
most common presentation of papillary fibroelastoma
[26]. This tumor has a predilection for the left side of
the heart: the aortic valve is the predominant site involved, followed by mitral leaflets. Grossly, papillary
fibroelastoma resemble a “sea anemone”. This tumor
usually has a gelatinous membrane on the surface and a
stalk with multiple delicate papillary projections, best
appreciated by immersing the specimen in water [27].
Microscopically, it is characterized by a collection of
avascular fronds of dense connettive tissue lined by
endothelium and may arise from any endocardial surface. Embolic fragments may originate from the tumor
itself and this occurs because of its very friable and soft
texture, or from surface formation of platelet and fibrin
thrombi [28]. Most are solitary and small, some are mobile and appear more likely to give rise to embolism.
Tumor mobility has been described to be an independent predictor of death or non-fatal embolization [29].
Echocardiography is the preferred means for evaluation
of papillary fibroelastomas [30]. Due to their small size
generally 0.5 to 2.0 cm in diameter and their valvular involvement, papillary fibroelastoma may be difficult to
distinguish from valvular vegetation. For this reason,
clinical informations, laboratory tests and blood cultures
are extremely important for differential diagnosis. The
differential diagnosis includes the presence of mixoma,
lipoma, rhabdomyoma or amorphous tumors. Since
symptomatic papillary fibroelastoma carries a definite
risk of severe complications, aggressive surgical management is recommended, irrespective of the tumor’s size
or the patient’s symptoms [26], the successful complete
resection of the papillary fibroelastoma is curative and
the long-term postoperative prognosis is excellent. The
patients who are not surgical candidates could be offered
long-term oral anticoagulation, although non randomized controlled data are available on its efficacy.

Conclusions
Echocardiography transthoracic and transesophageal are
essential investigations in young patients with stroke.
Differential diagnosis of cardiac masses requires clinical
informations, laboratory tests, blood cultures and appropriate use of imaging modalities. Papillary fibroelastoma
is a potential cause of embolic stroke in the young. The
prompt surgical excision of papillary fibroelastoma is
curative and the long-term postoperative prognosis is
excellent.

Page 4 of 5

Acknowledgements
Not applicable.
Funding
This study received no external funding.
Availability of data and materials
All data are in the medical record of the patient.
Authors’ contributions
All the authors have made substantial contributions in data interpretation,
and revised the manuscript critically. All authors read and approved the final
manuscript.
Competing interests
The authors declare that they have no competing interest.
Consent for publication
Written informed consent for publication of his clinical details and clinical
images was obtained from the patient.
Ethics approval and consent to participate
Not applicable (This is a Clinical Case Report and not a Study).

Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in
published maps and institutional affiliations.
Author details
1
Department of Cardiology, Ospedale dell’Angelo, Venezia-Mestre, Italy.
2
Department of Internal Medicine, Angiology Unit, Ospedale dell’Angelo,
Venezia-Mestre, Italy. 3Department of Cardiac Surgery, Ospedale dell’Angelo,
Venezia-Mestre, Italy. 4Department of Neurology, Ospedale dell’Angelo,
Venezia-Mestre, Italy.
Received: 29 November 2016 Accepted: 10 May 2017

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