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Case Report
Recurrent ischemic stroke caused by a cardiac tumor at a very unusual location: The importance of transesophageal echocardiography
Accidente isquémico vascular recurrente causado por un tumor cardiaco en una localización muy inusual: importancia de la ecografía transesofágica
María Fernanda Aguirre
Corresponding author
f_aguirre_c@hotmail.com

Corresponding author.
, Hugo Auquilla, María Nieves De Antonio, Begoña Bernal, Manuel Emilio Monguió, Guillermo Reyes Copa
Cardiac Surgery Department, Hospital Universitario de la Princesa, c/Diego de León 62, 28006 Madrid, Spain
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Cardiovascular magnetic resonance showing of a mass at the level of the ligament of Marshall &#40;space of atrial tissue separating the left atrial appendage &#40;LAA&#41; from the left upper pulmonary vein&#41;&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Cardiac papillary fibroelastoma &#40;PFE&#41; is a benign primary cardiac tumor with significant propensity for embolization&#46; Current reports indicate its increasing incidence&#44; being considered the most frequent benign cardiac tumor in adults&#44; probably due to the advent of transesophageal echocardiography &#40;TEE&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> Approximately 84&#37; of PFEs arise from valve surface&#44; especially in the aortic valve&#46; Non-valvular PFEs are very unusual&#44; and the location in the left atrium is exceedingly rare &#40;1&#46;6&#8211;2&#37;&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2&#44;3</span></a> To our knowledge&#44; there are only two cases reported to arise at the level of the ligament of Marshall&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Although most cases are asymptomatic and diagnosed incidentally&#44; symptomatic patients can present complications related to thromboembolic events&#44; more frequently transient ischemic attack and ischemic stroke&#44; which can be recurrent&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;4</span></a> Diagnosis is suspected during echocardiography&#44; but needs to be confirmed by histopathological examination&#46; A transesophageal echocardiogram is usually required&#44; due to its small size&#46; The only available curative treatment&#44; is complete surgical excision of the tumor&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;4</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">We present a case of a patient with recurrent ischemic stroke that presents with aphasia&#44; right hemiparesis and left oculocephalic deviation&#44; in which a mobile mass was observed at the level of the ligament of Marshall&#44; being an extremely rare location for this tumor&#46; Surgical resection was performed uneventfully and histopathologic examination confirmed the diagnosis of PFE&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Case report</span><p id="par0020" class="elsevierStylePara elsevierViewall">A 59-year-old woman arrived at the emergency department with acute onset of aphasia&#44; right hemiparesis and left oculocephalic&#46; Her medical history included smoking&#44; dyslipidemia and an ischemic stroke 4 month prior to admission with complete functional recovery&#44; in which an electrocardiogram &#40;EKG&#41;&#44; transthoracic echocardiography &#40;TTE&#41; and a twenty-four-hour EKG Holter monitoring were performed&#44; without encountering the embolic source of the stroke&#46; No usual risk factors for stroke were present&#44; such as hypertension&#44; diabetes or thrombophilia&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">She had no symptoms of cardiac illness&#44; and the clinical cardiovascular examination was unremarkable&#46; An EKG showed normal sinus rhythm and a right-bundle branch block not reported previously&#46; Chest radiography and laboratory results were within normal limits&#46; A cerebral computed tomography angiogram demonstrated ischemic lesions on the left middle cerebral artery territory and an emergent endovascular reperfusion was performed by thrombectomy without complications&#46; Neurologic evolution was favorable&#44; persisting mixed aphasia&#46; A TTE was performed showing only thickening of the free edge of the aortic valve veils generating mild aortic insufficiency&#44; but no cardiac mass was observed&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Five days after admission and without new symptoms&#44; a control cerebral computed tomography revealed a new established ischemic lesion in the left parahippocampal gyrus and hemorrhagic transformation in the left frontal operculum&#46; Due to high suspicion of cardiac source of emboli&#44; a transesophageal echocardiography &#40;TEE&#41; was performed revealing a mobile&#44; echogenic&#44; 20<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>15<span class="elsevierStyleHsp" style=""></span>mm pedunculated mass adhered to the ligament of Marshall &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; An MRI was also performed for better characterization of the mass &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">The patient underwent complete surgical excision of the mass through minimally invasive approach &#40;right anterior thoracotomy&#41;&#44; with cardiopulmonary bypass &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; Complete resection was confirmed by intraoperative TEE&#46; Posterior histopathologic examination confirmed the diagnosis of PFE &#40;<a class="elsevierStyleCrossRefs" href="#fig0020">Figs&#46; 4 and 5</a>&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia><elsevierMultimedia ident="fig0025"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">Postoperative course was uneventful with progressive neurological improvement&#44; persisting very mild dysphasia as a sequela&#46; There was no evidence of residual mass on the TTE performed 1 week after the intervention&#44; being discharged five days after surgery&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Discussion</span><p id="par0045" class="elsevierStylePara elsevierViewall">Primary cardiac tumors are very rare&#44; with an incidence ranging from 0&#46;0017&#37; to 0&#46;28&#37;&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;4</span></a> Myxomas were traditionally thought to be the most common&#44; nevertheless most of the supporting data was derived from autopsy series&#44; reflecting a probable underdiagnosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2&#44;4</span></a> Recent studies report the PFE as the most frequent primary cardiac tumor in adults&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> In a study that was made in a reference center by Tamin et al&#46;&#44; the frequency of PFE in patients undergoing TTE was greater than cardiac myxoma at a rate of approximately 2&#58;1&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a> The incidence is increasing probably due to higher quality of image with TEE&#44; improvements in technology&#44; enhanced awareness of PFE&#44; and an aging population&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">PFEs have high thromboembolic rate due to either the tumor itself or from adherent thrombi localized within the fronds&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;2&#44;4</span></a> Although most cases are asymptomatic and diagnosed incidentally&#44; almost half of patients with symptomatic PFEs&#44; presents as a transient ischemic attack or stroke &#40;42&#8211;53&#37;&#41;&#44; which can be specifically recurrent&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#44;5</span></a> Tumor mobility has been reported as an independent risk factor for embolization&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> Thorough etiologic work-up after a stroke is crucial to identify the underlying cause&#46; In a systematic review of international clinical practice guidelines in acute ischemic stroke<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a>&#44; the importance of the echocardiography is highlighted&#44; especially when a cardiac source is suspected&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">There are no current guidelines&#44; protocols of conservative or surgical treatment&#44; nor specific indication of anticoagulation therapy&#46; Tamin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> reported that patients without surgical intervention after diagnosis&#44; presented an incidence of 8&#46;9&#37; for having recurrent stroke&#44; with incremental risk over time&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2&#44;8</span></a> Sun et al&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> also observed high incidence &#40;6&#37;&#41; on presenting a thromboembolic event after diagnosis of PFE&#46; All of this data supports the indication for surgery in patients with suspected PFE&#44; for an accurate diagnosis and to avoid embolism&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">In almost a quarter of patients&#44; the diagnosis is obtained by TEE&#44; which was not observed by ETT&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> There are several studies describing the importance of cardiac imaging&#44; including 3D TEE or cardiac magnetic resonance&#44; for better characterization of the tumor&#44; relationships with surrounding tissues&#44; formulation of surgical approach&#44; and for the differential diagnosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3&#44;5&#44;9</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">The only curative treatment is complete surgical excision of the tumor&#44; with high likelihood of valve preservation&#44; especially if the patient is symptomatic and the tumor is on the left side of the heart&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> Surgery improves prognosis and reduces the risk of stroke substantially&#44; especially when performing primary resection of the tumor &#40;98&#37;&#41;&#46; Short- and long-term prognosis after surgical removal is excellent&#46;<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4&#44;10</span></a></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conclusions</span><p id="par0070" class="elsevierStylePara elsevierViewall">Cardiac tumors&#44; like papillary fibroelastoma&#44; should be considered as a differential diagnosis&#44; even in unusual locations&#44; after an ischemic stroke&#46; PFE has significant risk for recurrent thromboembolic events and should be adequately diagnosed&#46; The gold standard is the TEE&#44; which should be performed because of its higher sensitivity&#46; It is important to emphasize the extremely rare location of our patient&#39;s tumor&#46; Complete surgical excision as soon as possible is the treatment of choice&#44; because of the high risk of unpredictable embolization&#46; Surgery reduces the risk of recurrent neurologic events and increases long-term postoperative prognosis&#46;</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Ethical considerations</span><p id="par0090" class="elsevierStylePara elsevierViewall">The patient gave her consent for clinical information relating to this case to be reported in a medical publication&#46;</p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Conflict of interest</span><p id="par0095" class="elsevierStylePara elsevierViewall">The authors declare they have no conflict of interest&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Papillary fibroelastoma is a benign cardiac tumor generally found on valve surfaces&#44; although involvement of a cardiac chamber endocardium has been rarely reported&#46; They are usually asymptomatic&#44; but embolic events are highly associated presenting with neurological clinic&#46; We present a case of a patient with recurrent stroke presenting with aphasia&#44; right hemiparesis and left oculocephalic deviation&#44; in which a mobile mass was observed in the transesophageal echocardiography at the ligament of Marshall&#44; being a very unusual location for the tumor&#46; Surgical resection was performed uneventfully and histopathological examination confirmed the diagnosis&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El fibroelastoma papilar es un tumor cardiaco benigno que se encuentra generalmente en la superficie valvular&#44; siendo muy infrecuente la afectaci&#243;n del endocardio de las c&#225;maras cardiacas&#46; Suelen ser asintom&#225;ticos&#44; aunque eventos emb&#243;licos est&#225;n muy asociados manifest&#225;ndose con cl&#237;nica neurol&#243;gica importante&#46; Presentamos el caso de una paciente con ictus emb&#243;lico recurrente que presenta afasia&#44; hemiparesia derecha y desviaci&#243;n oculocef&#225;lica izquierda&#44; en la que se observ&#243; en el ecocardiograma transesof&#225;gico una masa m&#243;vil a nivel del ligamento de Marshall&#44; una localizaci&#243;n muy infrecuente del tumor&#46; Se realiz&#243; resecci&#243;n quir&#250;rgica completa y sin incidencias&#44; obteniendo posteriormente el diagn&#243;stico histopatol&#243;gico de fibroelastoma papilar&#46;</p></span>"
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