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Case Study
Tympanic Paraganglioma With Extension Into the Eustachian Tube and Nasopharynx: A Case Report
Paraganglioma timpánico con extensión a trompa de Eustaquio y nasofaringe: a propósito de un caso
Miriam Portero de la Torrea,
Corresponding author
miriam_portero@hotmail.com

Corresponding author.
, Rafael Sánchez Gutiérrezb, Inmaculada Gallardo Muñoza, Francisco A. Bravo Rodrígueza
a Servicio de Radiodiagnóstico, Hospital Universitario Reina Sofía, Córdoba, Spain
b Servicio de Otorrinolaringología, Hospital Universitario Reina Sofía, Córdoba, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Paragangliomas &#40;also known as &#8220;glomus&#8221; or &#8220;chemodectomas&#8221;&#41; are rare tumours derived from ectodermal cells of the neural crest&#46; The most frequent locations in the head and neck region are the carotid body&#44; jugular foramen&#44; vagus nerve and middle ear &#40;tympanic&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Tympanic paragangliomas are usually located immediately lateral to the middle ear promontory and may extend to the mastoid or the external auditory canal&#46; Rare extensions to the Eustachian tube have also been reported&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#8211;5</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">We report the case of a recurrent tympanic paraganglioma extending to the Eustachian tube and nasopharynx&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Clinical Case</span><p id="par0020" class="elsevierStylePara elsevierViewall">The patient was a 62-year-old female suffering recurrent epistaxis of 1-month evolution&#46; Four years earlier she had been intervened&#44; through left mastoidectomy&#44; due to a left tympanomastoid paraganglioma with a millimetric component in the Eustachian tube which was controlled&#46; During those 4 years the patient remained asymptomatic &#40;even missing some of the scheduled reviews&#41; until the onset of bleeding episodes&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">We performed a computed tomography &#40;CT&#41; scan with intravenous contrast&#44; which revealed a hypervascular mass in the left nasopharynx&#44; extending to the Eustachian tube and middle ear and eroding the bony walls &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Diagnostic angiography showed a hypervascular lesion with 3 compartments&#58; 1 in the middle ear&#44; irrigated by the inferior tympanic artery &#40;ascending pharyngeal branch&#41;&#44; another in the petrous apex&#44; irrigated by the caroticotympanic artery &#40;a branch of the internal carotid artery&#41; and another with caudal extension&#44; irrigated by branches of the ascending pharyngeal and accessory meningeal arteries &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">The findings were indicative of tympanic paraganglioma&#44; extending into the Eustachian tube and nasopharynx&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">In a second therapeutic stage&#44; we conducted a preoperative therapeutic angiography in order to reduce the risk of bleeding during surgery&#46; We closed the vascular supply from the middle meningeal branches&#44; internal maxillary&#44; stylomastoid and pharyngeal trunk of the left ascending pharyngeal artery by embolisation with Contour<span class="elsevierStyleSup">&#174;</span> polyvinyl alcohol particles of 150&#8211;250 microns &#40;Boston Scientific&#44; Cork&#44; Ireland&#41; and fibrous coils of 2&#215;10<span class="elsevierStyleHsp" style=""></span>mm &#40;Boston Scientific&#44; Cork&#44; Ireland&#41;&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Subsequently&#44; through endoscopic sinus surgery &#40;a less invasive technique than the external approach&#41;&#44; we performed laser ablation of the nasopharyngeal tumour and excised the tumoural portion located in the middle ear and Eustachian tube &#40;this resection was partial because the radical treatment entailed unassumable sequelae&#41;&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">The anatomopathological diagnosis was paraganglioma&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">After the intervention&#44; the patient underwent radiosurgery of the nasopharyngeal component&#46; In the last control CT scan &#40;2 years after surgery&#41;&#44; this component had become reduced to half its original size and the patient remained asymptomatic&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0060" class="elsevierStylePara elsevierViewall">Paragangliomas are rare tumours&#46; The peak age of onset is between the fifth and sixth decades of life&#44; with a higher prevalence among women&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Paragangliomas have capsules with septa and a stroma composed of sinusoidal spaces which are anastomosed together&#44; thus giving them their characteristic hypervascularity with pathognomonic arteriographic signs and haemorrhagic character &#40;which justifies preoperative embolisation&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Tympanic paragangliomas originate from glomus bodies of the cochlear promontory&#44; along the tympanic branch of the glossopharyngeal nerve &#40;nerve of Jacobson&#41;&#46; Examination usually reveals a hypotympanic or mesotympanic mass&#44; with the most common symptoms being pulsatile tinnitus and unilateral hearing loss&#46; CT and magnetic resonance imaging &#40;MRI&#41; scans show a mass in the medial wall of the middle ear&#44; which may extend to the mastoid or external ear canal&#46; Angiography shows characteristic findings&#44; revealing a hypervascular mass with compartmentalised vascularisation by specific hypertrophied arteries&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">The differential diagnosis should include undifferentiated carcinoma&#44; which shows less enhancement after contrast administration&#44; haemangioma and Kaposi sarcoma&#44; which are vascular tumours&#44; although there are no reported cases of Eustachian tube extension&#44; and juvenile nasopharyngeal angiofibroma&#44; which is a highly vascular tumour&#44; almost exclusively found in adolescent males&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">Only 4 cases of tympanic paraganglioma extending into the Eustachian tube and nasopharynx have been published&#44;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#8211;5</span></a> all of them occurring in women aged between 50 and 65 years and beginning with epistaxis&#44; as in our patient&#46; In the cases described&#44; the diagnosis was obtained by CT<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#8211;5</span></a> and&#47;or MRI&#44;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#8211;4</span></a> supplemented in some cases by angiography<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;5</span></a> and biopsy&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Treatment was surgical resection<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> or radiotherapy<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#8211;4</span></a> &#40;1 patient underwent ligation of the external carotid artery prior to radiotherapy<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a>&#41;&#46; Only 1 of these cases underwent preoperative embolisation by angiography&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Our case illustrates a very unusual extension of tympanic paraganglioma&#44; with characteristic findings on CT scans and angiography&#46; The latter was useful in both diagnosis and preoperative treatment of the lesion&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">In summary&#44; the diagnosis of paraganglioma should be considered in patients with epistaxis and CT and&#47;or MRI findings of hypervascular lesions in the middle ear extending to the Eustachian tube and nasopharynx&#46; In such cases&#44; angiography will enable better characterisation of the lesion and its vascular supply&#46; Embolisation prior to biopsy or surgical resection will diminish the risk of uncontrollable bleeding&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflict of Interests</span><p id="par0095" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "titulo" => "Abstract"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Tympanic paragangliomas usually present as a vascular middle ear mass&#44; with the most common presenting symptoms being pulsatile tinnitus and hearing loss&#46; We report an unusual case of a recurrent tympanic paraganglioma extending along the Eustachian tube and nasopharynx&#44; presenting with recurrent epistaxis&#46;</p>"
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      "es" => array:2 [
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        "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Los paragangliomas timp&#225;nicos suelen presentarse como una masa hipervascular en o&#237;do medio&#44; siendo los s&#237;ntomas de presentaci&#243;n m&#225;s frecuentes ac&#250;feno puls&#225;til e hipoacusia&#46; Presentamos un caso infrecuente de recidiva de paraganglioma timp&#225;nico con extensi&#243;n a trompa de Eustaquio y nasofaringe&#44; que debut&#243; con epistaxis recurrente&#46;</p>"
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        "nota" => "<p class="elsevierStyleNotepara">Please cite this article as&#58; Portero de la Torre M&#44; et al&#46; Paraganglioma timp&#225;nico con extensi&#243;n a trompa de Eustaquio y nasofaringe&#58; a prop&#243;sito de un caso&#46; Acta Otorrinolaringol Esp&#46; 2012&#59;63&#58;314&#8211;7&#46;</p>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">CT scan with intravenous contrast&#46; The axial &#40;A&#41; and coronal &#40;B&#41; sections show a hypervascular mass located in the left nasopharynx &#40;white arrow&#41;&#44; Eustachian tube &#40;black arrow&#41; and middle ear&#46; &#40;C&#41; Reconstruction of the lesion in the major axis &#40;white arrow&#41;&#44; showing its extension from the middle ear to the nasopharynx&#46;</p>"
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Digital subtraction angiography&#46; &#40;A&#41; Left internal carotid arteriography&#46; There is a compartment of the lesion &#40;black arrow&#41; which is irrigated by the caroticotympanic branch of the artery&#46; &#40;B&#41; and &#40;C&#41; Left external carotid arteriography&#46; This image shows the other 2 compartments&#58; one irrigated by the inferior tympanic branch &#40;black arrow&#41; of the ascending pharyngeal&#44; and another&#44; larger one&#44; irrigated by pharyngeal branches of the ascending pharyngeal artery and branches of a hypertrophied&#44; accessory meningeal artery &#40;white arrow&#41;&#46; &#40;D&#41; Anteroposterior view showing the extension of the lesion&#46;</p>"
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                      "titulo" => "Nasopharyngeal extension of glomus tympanicum&#58; an unusual clinical and imaging manifestation"
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                      "titulo" => "Unusual eustachian tube mass&#58; glomus tympanicum"
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es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos