Cardiovascular Pathology 40 (2019) 65–67

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Cardiovascular Pathology

Clinical Case Report

Brainstem stroke caused by left atrial cardiac papillary fibroelastoma:
an increasingly recognized rare cause of stroke
Luke Bonavia ⁎, Justin Jackson, Jennifer Pui-Yee Law, Michael Yii, Dhaval Joshi
Albury Base Hospital NSW, Epworth Eastern Hospital and Melbourne Pathology Victoria, Australia

a r t i c l e

i n f o

Article history:
Received 13 January 2019
Received in revised form 30 January 2019
Accepted 31 January 2019
Keywords:
Cardiac papillary fibroelastoma
Embolic stroke
Older adults
Atrium
Transesophageal echocardiography

a b s t r a c t
Cardiac papillary fibroelastoma is a rare but increasingly recognized cause of embolic stroke that is prevalent in the older population and requires prompt surgical management. We report an unusual case of left
atrial appendage cardiac fibroelastoma in a 76-year-old gentleman who presented with left internuclear
ophthalmoplegia and ataxia, with corresponding diffusion-weighted imaging on magnetic resonance imaging of the brain. This case illustrates the importance of echocardiographic imaging in the workup of cardioembolic stroke in the older adult population in the acute setting.
Crown Copyright © 2019 Published by Elsevier Inc. All rights reserved.

1. Case
We report a case of a 76-year-old gentleman presenting with posterior territory infarcts caused by cardiac papillary fibroelastoma (CPF) of
the left atrial appendage. He presented initially with sudden severe
vertigo, diplopia, and left-sided ataxia while reading the newspaper on
the morning on admission. This is on a background of hypertension and
dyslipidemia. Examination on presentation indicated a left internuclear
ophthalmoplegia with a left sixth cranial nerve palsy. Past pointing was
noted on the left arm, and corresponding abnormal heel–shin test was
identified on the left leg. Mild right arm weakness was also noted, and
sensory examination was normal. Notably heart sounds were dual with
no murmurs.
He was thrombolyzed within 2 h of symptom onset. His computed
tomography (CT) angiogram did not show any basilar or vertebral
occlusion or dissection. Magnetic resonance imaging (MRI) of the
brain indicated diffusion restriction in the left medial midbrain and left
cerebellar regions consistent with clinical findings (Figs. 1 to 5). There
was no evidence of atrial fibrillation on ECG or telemetry.
Transthoracic echocardiogram indicated a 24×18×20-mm isoechoic,
solid, highly mobile mass attached to the anterior left atrial wall below
the anterior mitral leaflet. Transesophageal echocardiogram confirmed
the presence of a spherical mobile mass attached to the orifice of the left
atrial appendage (Figs. 6 and 7, Supplementary Video 1 “Supplementary
video 1: TOE video.avi”).

The authors report no conflict of interest.
⁎ Corresponding author.
E-mail address: ljbonavia@gmail.com (L. Bonavia).

https://doi.org/10.1016/j.carpath.2019.01.007
1054-8807/Crown Copyright © 2019 Published by Elsevier Inc. All rights reserved.

Our patient was transferred to a tertiary center for resection. Under
cardiopulmonary bypass and cardioplegic arrest, a fragile 22-mm round
pendunculated mass was excised from the ostium of the left atrial
appendage. The left atrial appendage was then oversewn. An incidental
patent foramen ovale (PFO) was noted intraoperatively and also
oversewn. Coronary artery bypass was performed for coincidental coronary artery disease.
Histopathologic analysis of the 22×18×12-mm ovoid mass however
noted branching of papillary fronds lined with epithelial cells and
collagenous core with focal areas of myxoid change consistent with a
papillary fibroelastoma (Figs. 8 and 9).
Our patient's complex ophthalmoplegia resolved by day 3 into
admission; however, his left cerebellar ataxia persisted and required
rehabilitation. After 3 months postexcision, there have been no further
cerebrovascular events.

2. Discussion
CPFs are benign primary cardiac tumors and a rare but increasingly
recognized cause of cardioembolic stroke and transient ischemic attack.
While previously thought to be less common than atrial myxomas, with
increasing echocardiology surveillance, CPFs are now the most common
benign cardiac tumor [1,2]. CPFs commonly have a predilection for aortic
and mitral valves [3] and are rarely tethered to the atrial epithelium. Left
atrial CPFs are exceedingly rare, estimated to be between 1.3% and 6.1% of
all CPFs as described by large retrospective studies [3–6]. Unlike atrial
myxomas, they have not been reported to present with a systemic prodrome [1]. A recent retrospective analysis of 67 cases of stroke in CPF
demonstrated anterior circulation infarcts in 93% of cases [7].

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L. Bonavia et al. / Cardiovascular Pathology 40 (2019) 65–67

Fig. 5. Normal MRI angiogram vessel construct.

Figs. 1 and 2. MRI diffusion-weighted imaging and corresponding ADC image of ischemia
within midbrain and base of cerebellum.

They have fragile, frond-like morphology and a collagenous core and
are pedunculated in about half of cases [3,8]. When submerged in saline,
they resemble sea anemones [9]. Of the few cases of left atrial CPFs
described, they were resected from the superior and inferior aspects
of the pulmonary veins, atrial appendage, and septum, and one case
tethered to cor triatrium [9–14]. They average between 10 mm and
30 mm in diameter and can be identified on echocardiography [5].
Sensitivity and specificity of transthoracic echocardiography, however,
reduce with sizes less than 20mm3. Echocardiographic workup should
therefore include transesophageal echocardiography in suspected cases.
Unlike atrial myxomas, CPFs have a mean age of 60 years at diagnosis
without a predominant gender [1,3]. Out of the cases of left atrial CPF,
patient ages ranged from 55 to 80 [9,12–17]. However, valvular CPFs
have been reported to occur in patients less than 30 years of age
[18,19]. Posterior circulation infarct from a left atrial CPF has been
described once [14]. A recent retrospective analysis of 67 cases of stroke
in CPF demonstrated anterior circulation infarcts in 93% of cases [7].
CPFs are thought to be acquired rather than inherited, with possible
contributing factors including epithelial hyperproliferation from hemodynamic trauma, organized thrombi, and underlying genetic predisposition
[3]. Prior cardiac procedures, radiation therapy, and chronic CMV endocarditis have also be proposed as contributing factors [3]. None of these
factors are present in our case. Given their fragile composition, segments
of the tumor may dislodge and contribute to systemic embolization.
Additionally, the mucopolysaccharide and hyaluronic-acid-rich frond
surface may harbor thrombi that may embolize. In a retrospective study

of 725 cases of CPF, thrombi were noted on 19 cases perioperatively,
and 3 cases of recurrent strokes were noted despite warfarinization [20].
Large retrospective analyses estimate stroke or transient ischemic
attack risk in CPF to be between 13.5% and 53.6% [2,4–6,8,20]. Recurrence
of stroke in nonresected tumors at diagnosis was estimated to be
between 16% and 24% with mean follow-up of 31 months [5,20]. Sun
et al., however, noted a recurrence rate of over 50% with a median
follow-up of 130 days [8]. Tamin et al. recently demonstrated a recurrence of stroke in nonresected CPF to be 6% in 12 months and 13% in
5 years [2]. Cases of stroke due to valvular or nonvalvular CPF have
been reported to respond to thrombolysis, such as this case [7]. After
resection, stroke recurrence rates are low [2,4,8], and survival rates are
excellent [21]. However, it is important to note that while stroke recurrence is significantly reduced after resection, background cerebrovascular
risk warrants consideration [2]. There is no consensus on appropriate
anticoagulation postresection, although anticoagulation is warranted if
surgery is not an option [3].
It is important to identify that our patient does have known cerebrovascular risk factors of hypertension and dyslipidemia [22]. However,

Figs. 6 and 7. Atrial views of the mobile appendicular mass noted on transesophageal
echocardiography.

Figs. 3 and 4. MRI diffusion-weighted imaging and corresponding ADC image of ischemia
within cerebellum.

Figs. 8 and 9. Histopathology of the ovoid mass at 2× and 4× magnification. Note the
frond-like appearance, peripheral epithelial cells, and collagenous core (thanks to
Dr. Dhaval Joshi MBBS FRCPA, Melbourne Pathology, Australia).

L. Bonavia et al. / Cardiovascular Pathology 40 (2019) 65–67

the multiembolic nature of the infarcts suggests a cardiac or proximal
arterial source. A normal CT and MRI angiogram makes a proximal arterial source unlikely (Fig. 5).
Furthermore, a concomitant PFO was identified intraoperatively. PFOs
are found in 40% of the population, and risk of stroke is not well
established [23]. Given the presence of a CPF with its recognized embolic
stroke risk as described above, it is more likely that our patient's stroke
was attributable to the identified atrial source rather than his cerebrovascular risk factors alone or his PFO.
This case highlights the growing recognition of CPF as a rare but
important cause of cardioembolic stroke in the older population.
Differential diagnosis of a left atrial mass should include a CPF. The
risk of stroke recurrence for a given diagnosis is high, and prompt surgical resection is recommended.
Supplementary data to this article can be found online at https://doi.
org/10.1016/j.carpath.2019.01.007.

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