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Juvenile nasopharyngeal angiofibroma with orbital extension
Angiofibroma nasofaríngeo juvenil con extensión orbitaria
A. Hervás Ontiverosa,
Corresponding author
anheront@gmail.com

Corresponding author.
, E. España Gregoria, L. Climent Vallanoa, S. Rivas Roderob, A. Alamar Velázquezc, J.A. Simal Juliánd
a Unidad docente de Oftalmología, Departamento de Cirugía, Universidad de Valencia, Hospital Universitario La Fe, Valencia, Spain
b Departamento de Anatomía Patológica, Hospital Universitario La Fe, Valencia, Spain
c Departamento de Otorrinolaringología, Hospital Universitario La Fe, Valencia, Spain
d Departamento de Neurocirugía, Hospital Universitario La Fe, Valencia, Spain
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21 years of age&#44; without relevant history&#44; presented with left exophthalmos and diplopia in extreme gaze positions with 2 weeks evolution&#44; without clinic of epistaxis or nasal obstruction &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Visual acuity was 20&#47;20 in both eyes and intraocular pressure &#40;IOP&#41; 24<span class="elsevierStyleHsp" style=""></span>mmHg in left eye &#40;LE&#41; without limitations in ocular movement&#46; Ocular fundus did not reveal alterations&#46; Cerebral-orbitary magnetic resonance &#40;MR&#41; exhibited a highly vascularized left ethmoidal lesion with prominent vascular structures&#46; Orbitary extension was observed with intra- and extraconal components which displaced the internal and inferior rectus&#46; Ethmoidal cells exhibited bone destruction and extension toward the anterior cranial fossa&#44; associating contrast capturing in the adjacent dural meningeal zone &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Arteriography revealed a highly vascularized tumor at the ethmoidal level&#44; discarding the presence of arteriovenous malformations &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; The remainder of the extension study was negative&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Initially and in the light of the clinical behavior and radiological findings an anterosuperior biopsy was performed&#44; with the result of abundant bleeding&#46; For this reason it was decided to postpone the intervention in order to approach the lesion through the anterior and nasal endoscopic path&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The lesion biopsy exhibited the presence of capillary and venous blood vessels with irregular muscular walls which in addition were dilated and covered by prominent endothelium&#46; Said vessels were surrounded by spindle cells with oval nucleii but without atypical mitosis&#46; Associated inflammatory infiltrate was found which produced necrosis and destruction in the ethmoid bone tissue with extension toward the adjacent sinus mucosa and intracranially toward the dura mater &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46; The immunohistochemical study revealed angiomatous and fibroblastic properties with positive staining against smooth muscle vimentin&#44; CD34&#44; bcl-2&#44; CD99&#44; actin and myosin&#46;</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">The sample analysis enabled the JNA diagnostic&#46; Surgical approach was decided by means of anterosuperior and endoscopic orbitotomy&#46; When the region was accessed&#44; significant bleeding was evidenced and the tumor was partially resected&#46; One week post-surgery MR revealed an intra-orbit nodule suggesting tumor remains and persistence of contrast capture at the frontal dural level &#40;<a class="elsevierStyleCrossRef" href="#fig0025">Fig&#46; 5</a>&#41;&#46; Subsequently&#44; a left orbit ethmoidal endonasal approach was decided to achieve almost complete removal of the mass and medial wall reconstruction &#40;LactoSorb<span class="elsevierStyleSup">&#174;</span> Plating System&#44; Biomet Microfixation&#44; Jacksonville&#44; FL&#44; USA&#41;&#46; Postop&#46; MR revealed a small left frontal post-surgery collection with small nodular capture at the meningeal level &#40;<a class="elsevierStyleCrossRef" href="#fig0030">Fig&#46; 6</a>&#41;&#46; Proptosis exhibited significant improvement&#44; and ocular parallelism was maintained with discreet exophthalmos without diplopia&#46; IOP in the LE improved to 16<span class="elsevierStyleHsp" style=""></span>mmHg&#46;</p><elsevierMultimedia ident="fig0025"></elsevierMultimedia><elsevierMultimedia ident="fig0030"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">Although the exact nature of JNA is not known&#44; the first histological studies suggested it could originate in fibrocartilage&#44; periosteum or embryonary fascia&#46; Subsequently&#44; Liang et al&#46; reviewed a series of 25 tumors which concluded a vascular origin&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">JNA is a benign neoplasia with local malignity characteristics due to its ability to invade adjacent areas&#46; In the present case&#44; onset comprised orbit extension expression without nasal clinic&#44; exhibiting diplopia in extreme positions due to compromised space&#46; In this way&#44; the tumor invaded the posterior portion of the orbits through the media wall from the ethmoidal <span class="elsevierStyleItalic">lamina papyracea</span>&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">The diagnostic is based on clinical findings and imaging tests which also determine the tumor extension&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> A differential diagnostic must be performed with other types of nasopharyngeal tumors &#40;choanal polyp&#44; adenoid hyperplasia&#41;&#44; syphilis and nasopharyngeal tuberculosis&#44; craniopharyngioma or lymphoma&#44; among others&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">JNA is a therapeutic problem due to the risk of abundant bleeding and high recurrence rates&#46; In general&#44; surgical treatment is preferred with or without presurgery embolization&#46; In recent years&#44; endoscopic surgery is the treatment of choice as it allows satisfactory resections with the advantage of being able to see several angles of adjacent structures&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">In the present case&#44; the endonasal approach facilitated control of the intra-surgery bleeding and satisfactory tumor resection&#46; Radiotherapy allowed control of the disease in highly advanced stages&#46; At present&#44; after a follow-up of one year&#44; the patient has not exhibited relapses of the disease and has been referred to our practice every 6 months&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conflict of interest</span><p id="par0060" class="elsevierStylePara elsevierViewall">No conflict of interest has been declared by the authors&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Clinical case</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">The case of a 21-year-old male with a history of left proptosis and diplopia of two weeks of onset is presented&#46; The MRI showed an ethmoid-orbital vascular lesion with anterior skull base invasion and orbital extension&#46; Biopsy of the ethmoid confirmed fibrovascular tissue&#44; which supported the diagnosis of angiofibroma&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Discussion</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">It is a benign neoplasm with local characteristics of malignancy due to its ability to invade adjacent areas&#46; In this case&#44; the debut presented with manifestations of orbital extension&#46; A broad and multidisciplinary approach is needed in order to improve prognosis&#46;</p></span>"
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Vision recovery following nasopharyngeal angiofibroma excision"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "J&#46;A&#46; Patrocinio"
                            1 => "L&#46;G&#46; Patrocinio"
                            2 => "L&#46;P&#46; Martins"
                            3 => "A&#46;R&#46; da Cunha"
                          ]
                        ]
                      ]
                    ]
                  ]
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                        "fecha" => "2002"
                        "volumen" => "29"
                        "paginaInicial" => "309"
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                        "link" => array:1 [
                          0 => array:2 [
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                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0035"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Juvenile nasopharyngeal angiofibroma&#58; The routes of invasion"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "L&#46;U&#46; Sennes"
                            1 => "O&#46; Butugan"
                            2 => "T&#46;G&#46; S&#225;nchez"
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                        ]
                      ]
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              ]
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              "identificador" => "bib0040"
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              "referencia" => array:1 [
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                  "contribucion" => array:1 [
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                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
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Article information
ISSN: 21735794
Original language: English
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2017 December 5 1 6
2017 November 21 2 23
2017 October 19 3 22
2017 September 21 2 23
2017 August 15 2 17
2017 July 19 5 24
2017 June 23 4 27
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2017 April 30 6 36
2017 March 36 65 101
2017 February 49 2 51
2017 January 57 2 59
2016 December 70 3 73
2016 November 39 3 42
2016 October 51 5 56
2016 September 48 4 52
2016 August 28 1 29
2016 July 24 4 28
2016 June 26 3 29
2016 May 19 2 21
2016 April 13 2 15
2016 March 23 6 29
2016 February 16 9 25
2016 January 15 4 19
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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