European Journal of Neurology 2000, 7: 449]453
CLINICAL CORRESPONDENCE

Mitral papillary fibroelastoma as a cause of cardiogenic embolic stroke:
report of two cases and review of the literature
J. Sastre-Garriga, C. Molina, J. Montaner, A. MauleoÂn, F. Pujadas, A. Codina and J. AÂlvarez-SabõÂn
Unitat Cerebrovascular, Servei de Neurologia, Hospital General i Universitari Vall d'Hebron
Keywords:

cerebral embolism and
thrombosis,
echocardiography, heart
neoplasms,
transoesophageal
Received 24 November 1999
Accepted 9 March 2000

Papillary fibroelastoma (PFE) is a rare benign tumour that attaches to the endocardial
surface, mostly on cardiac valves. Though usually asymptomatic, it can be the source
of several complications. To date, 49 cases have been reported of embolic stroke with a
PFE as the probable origin. Case reports: (i) a 39-year-old male presented with
ischemic embolic stroke; the presence of a PFE was assessed by means of
transoesophageal echocardiography and confirmed by pathological findings; (ii) a
32-year-old woman presented with sudden onset of left hemiparesis; a cardiogenic
embolic stroke was suspected, and a diagnosis of PFE was made based on
echocardiographic and pathological findings. In both cases, surgical excision of the
tumours was performed with no recurrences at follow-up. Two mechanisms can
explain the formation of emboli in PFE: dislodgement of the tumour leaves or fibrinplatelet aggregation on the endocardial surface of these leaves. Transthoracic
echocardiography may lead to the suspicion of a PFE, but transoesophageal
echocardiography is required for confirmation. Prompt surgical excision is indicated
in most cases. Anticoagulation is only recommended in situations of high surgical risk
and during the wait for surgery.

Background
Papillary fibroelastomas (PFE) are rare benign tumours; they make up 10% of all primary cardiac
tumours. They are usually located in cardiac cavities,
most often on valvular endocardium. They may be
differentiated from mixoma cordis by their predominant
location on heart valves and by their histopathological
features and echocardiographic appearance. They consist of multiple papilliform leaves which are joined
together in a short stalk attached to the endocardium
surface. These leaves are surrounded by hyperplastic
endocardial cells which are continuous with the
endocardial endothelium of the cardiac cavities (McAllister, 1991). Although mostly asymptomatic, they may
manifest clinically as sudden death, heart failure,
myocardial infarction, angina or embolic stroke. We
have found 49 cases reported, to date, of embolic stroke
in patients with a pathological demonstration of a PFE.
The embolic mechanism is not well known, but could be
explained either by the dislodgement of tumoral
fragments, or by the embolization of fibrin thrombi
raised at the papillary surface. We present two cases: a
Correspondence: Dr Jaume Sastre-Garriga, Hospital General de la
Vall d'Hebron, Unitat Cerebrovascular, Servei de Neurologia, Passeig
de la Vall d'Hebron 119]129, 08035 Barcelona (fax: + 93 274 62 55;
e-mail: jaumesg@bitel.es).

ã 2000 EFNS

39-year-old caucasian man who suffered from an
ischaemic stroke and a 32-year-old woman who
suffered from an ischaemic-haemorrhagic stroke. Both
ischaemic processes were due to a PFE, diagnosed by
means of transoesophagic and transthoracic echocardiography and confirmed pathologically.

Case studies
Case 1

A 39-year-old man, smoker of 40 cigarettes per day, exdrinker of 80 g/day, with no other cardiovascular risk
factors, presented with sudden left hemiparesis and
ipsilateral severe hemihypoesthesia and dysarthria. All
these symptoms resolved within 72 h of admission. He
was seropositive for hepatitis C virus and had been
diagnosed with chronic hepatitis three years before. He
was treated until one year ago with interferon-a and
ribavirine. Haematological, biochemical and routine
coagulation tests were normal. Protein C, protein S,
and antithrombin III levels were normal and activated
protein C resistance determination did not show any
abnormality. Syphilis and human immunodeficiency
virus serologies were negative. Electrocardiogram
(EKG) showed sinus rhythm and the thorax X-ray
was normal. Cranial computed tomography (CT)
performed four hours after the onset of symptoms

449

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J. Sastre-Garriga et al.

revealed cortico-subcortical isodensity with mild global
hypodensity of frontal and temporal right lobes. A
Doppler carotid ultrasound and transcranial Doppler
were normal. A 24-h EKG monitoring showed nonspecific sinus rhythm. Cranial magnetic resonance
imaging (MRI) revealed cortical acute infarction
located in the insulo-opercular right frontal zone,
partially affecting the superficial territory of the middle
cerebral artery. The angiographic MRI scans failed to
reveal esteno-occlusive lesions on intracranial arteries.
A transthoracic echocardiogram found an unclear
image of vegetation/thrombus over the posterior leaflet
of the mitral valve. A transoesophageal echocardiogram was therefore performed, revealing a mass
(8 3 9 mm) attached to the posterior leaflet of the
mitral valve, suggesting a PFE. Surgical excision of the
tumour with valve preservation was chosen as the best
therapy and was undertaken without complications.
The results of the pathological study were compatible
with PFE, showing multiple papillary leaves composed
of a central core of connective tissue (with positive
staining for elastic fibers) and lined by hyperplastic
endocardial cells (Fig. 1).
Case 2

A 32-year-old woman, smoker and former user of oral
contraceptives, with no other cardiovascular risk
factors, presented with sudden left faciobrachiocrural
hemiparesis. Her medical history was not remarkable.
Haematological, biochemical and routine coagulation
tests were normal. Protein C, protein S, and antithrombin III levels were normal and activated protein C
resistance determination did not show any abnormality.
A Doppler carotid ultrasound and transcranial Doppler
were normal. EKG showed sinus rhythm with signs of

Figure 2 A transthoracic echocardiogram found a rounded mass
(15 3 10 mm) attached to the septal leaflet of the mitral valve,
suggesting papillary fibroelastoma.

inferolateral necrosis and anterior hemi-blockade. A
CT brain scan, performed within the first 24 h of
symptoms, was normal. Another CT brain scan
performed three weeks later showed cortico-subcortical
hypodensity with diffuse areas of hyperdensity within
the right temporal lobe, compatible with subacute
haemorrhagic infarct. A transthoracic echocardiogram
found a rounded mass (15 3 10 mm) attached to the
septal leaflet of the mitral valve suggesting papillary
fibroelastoma (Fig. 2). Angiographic study showed
permeable coronary arteries and inferoapical akinesia
with normal systolic function of the left ventricle.
Surgical excision of the tumour with valve preservation
was chosen as the best therapy and was undertaken
without complications. The result of the pathological
study was compatible with PFE, showing multiple
papillary leaves composed of a central core of
connective tissue surrounded by endocardial hyperplastic cells.

Discussion

Figure 1 Histopathological study (haematoxilin-eosin) of surgical
fragment showing papillary structure of fibroelastoma. Using
specific techniques, positive staining for elastic fibers was found.

Primary cardiac valve tumours are very rare. Until
1995, a cumulative total of 134 in 128 patients had been
reported, 41 (31%) of which correspond to mitral valve
attachment. The most common histological type
reported is papillary fibroelastoma (117/132; Ryan
et al., 1995). Although usually an incidental finding in
echocardiographic or autopsy studies, some PFEs may
cause sudden death, heart failure, angina, acute
myocardial infarction and embolic stroke. Until 1988,
eight cases of embolic stroke were reported as a
complication of a PFE (Kasarskis et al., 1988). Since
1988, 41 cases with pathological demonstration, including ours, have been reported (Table 1). The mean age at

ã 2000 EFNS European Journal of Neurology 7, 449]453

Papillary fibroelastoma and stroke

451

Table 1 Papillary fibroelastoma and stroke: reported cases

Author
Yater (1931)*
Abu Nassar and Parker (1971)*
Fowles et al. (1981)*
Ong et al. (1982)*
Topol et al. (1986)
McFadden et al. (1987)
Kasarskis et al. (1988)
Gorton et al. (1989)
Ortiz de Murua et al. (1990)
Kroll et al. (1991)
Edwards et al. (1991)
Imamaki et al. (1993)
Gallo et al. (1993)
Schuetz et al. (1993)
RoldaÂn-torres et al. (1994)
Mann et al. (1994)
Ragni et al. (1994)
Colucci et al. (1995)
Pinelli et al. (1995)
Ryan et al. (1995)
Rubin et al. (1995)
Brown et al. (1995)

Shirota et al. (1996)
Muir et al. (1996)
Ni et al. (1996)
De Menezes et al. (1996)
Wolfe et al. (1997)
Shelh (1997)
Lund et al. (1997)
Al-M et al. (1998)
Mayer et al. (1998)
Pacini et al. (1998)
Conte et al. (1998)
Giannesini et al. (1999)
Sastre-Garriga et al.
(personal communication)

Age
of patient

Gender
of patient

TIA or previous
stroke

Territory of
infarction

70
54
35
71
27
41
43
52
77
68
25
(2 cases)
73
21
57
32
59
53
66
27
24
36
34
55
40
68
39
46
31
3
75
49
36
64
44
66
40
37
39
32

M
M
M
F
M
F
M
F
F
F
F
?
F
M
F
F
F
M
F
F
M
M
M
F
F
M
M
M
F
F
F
F
F
F
F
F
F
F
M
F

?
?
Yes
Yes
No
No
No
Yes
Yes
Yes
Yes
?
Yes
No
Yes
Yes
Yes
Yes
Yes
No
Yes
No
No
Yes
No
No
No
Yes
No
No
Yes
No
No
No
No
Yes
No
No
No
No

MCA left
MCA left
MCA left
MCA right
MCA left
MCA left
Only TIA
MCA left, MCA right, basilar
Only TIA
Only TIA
Only TIA
?
Only TIA
MCA left
MCA left
Ophthalmic artery
Only TIA
Only TIA
Only TIA
Basilar
MCA left
Only TIA
Ophthalmic artery
Only TIA
MCA left
Ophthalmic artery
MCA right
MCA left
MCA left
MCA left
MCA right
MCA right
MCA right
Only TIA
Basilar
Only TIA
MCA left
MCA right
MCA right
MCA right

MCA: middle cerebral artery; TIA: transient ischemic attack. *Taken from Kasarskis et al. (1988).

diagnosis was 46 years (3]77) and the median age was
43 years, with a slight female predominance in sex
distribution (35% men). In 45% of the cases the
ischaemic event (transient ischaemic attack ] TIA ]or
complete cerebral infarction) which led to diagnosis was
preceded by one or more TIA. Various clinical tableaux
led to diagnosis: infarction of left middle cerebral artery
(MCA) territory (33%), infarction of right MCA
territory (21%), TIA (31%), basilar artery territory
infarction (5%) and occlusion of retinal central artery
(8%). One patient, representing 3% of all cases,
presented with multiple infarcts of anterior and
posterior vascular brain territories. Emboli may arise

ã 2000 EFNS European Journal of Neurology 7, 449]453

from two different mechanisms: they might be due to
thrombi formation on the surface of papillary
structures, or to tumoral frond dislodgement (Conte
and Katz, 1998). The embolic capacity of this tumour is
reinforced by the demonstration of high intensity
Doppler signals in both carotid artery territories,
suggesting particulate embolism of cardiac origin. In
this case, these high intensity signals ceased after
tumour excision (Muir et al., 1996). On the other
hand, pathological studies have demonstrated the
presence of fibrin-platelet aggregates on the surface of
PFE leaves (Brown et al., 1995).
Regarding diagnosis, in a clinically suspected embolic

452

J. Sastre-Garriga et al.

stroke, one must perform a transthoracic echocardiography (TTE) which, on occasion, may fail to reveal the
presence of the tumour. The way to achieve the best
definition and to rule out multiple PFEs is to perform a
transoesophageal echocardiography (TEE). Transoesophageal echocardiography is also the best option in the
case of high suspicion and normal TTE (Shelh, 1997).
TEE has been also used as an intraoperatory guide to
excision (Topol et al., 1986). The echocardiographic
characteristic image (Hicks et al., 1996; Klarich et al.,
1997) is that of an excrescence mobile, digitiforme and
pediculated in one or more valvular leaflets, the
smallest being of millimetres and the largest around
five centimetres. These excrescences are attached to the
endocardium of cardiac native valves by short stalks.
They are located in the midportion of valvular surfaces,
away from the free margins. Their typical locations are
the atrial surface of mitral and tricuspid valves and the
ventricular surface of semilunar valves. PFEs have a
more defined acoustic interface than vegetations.
Nevertheless, in some instances, it is not possible to
make an exact diagnosis using only echocardiography,
and the clinical context and a pathological study will
confirm the suspicion. The histogenesis of PFE remains
controversial. Some authors consider PFE as a type of
organizing thrombus, while some others suggest a
hamartomatous origin. Rubin et al. (1995), using
immunohistochemical techniques, demonstrated active
participation of the vascular endothelial cells in the
formation of PFE, suggesting that PFEs are more than
an organizing thrombus. Whether PFE represents a
true neoplastic or a hamartomatous process is still to be
confirmed. Histologically, these benign tumours consist
of a central region of dense connective tissue surrounded by a layer of loose connective tissue which is
covered by endothelial cells. All of these components
are in continuity with those of normal endocardium.
Elastic fibers are always found in the loose connective
tissue layer, and are considered the PFE hallmark.
PFEs are thought to be distinguished from Lambl's
excrescences by composition and location (McAllister,
1991).
The first choice treatment of a symptomatic PFE is
surgical excision. It must be performed as soon as
possible to diminish the risk of early recurrences. In
high surgical risk patients the most appropriate
treatment option would be life-long oral anticoagulation (Pinelli et al., 1995). In asymptomatic
PFEs, the site of implantation would be the guide to
therapeutic choice. A decision on therapeutic choice
may be delayed when the PFEs are located in the right
cavities until the course is known. In asymptomatic
PFEs of left cavities, therapeutic choice must be
individualized, except for papillary fibroelastomas

attaching to the endocardium of the cardiac cavities.
Since these are more aggressive in behaviour, excision
would be the best choice (Yee et al., 1997). In most
cases, if the patient does not have a very high surgical
risk, surgical excision with valve preservation may be
systematically elected.

Acknowledgements
The authors thank Ms Julie Myers for linguistic
assessment.

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