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Rengel Ruiz, E. España Gregori" "autores" => array:3 [ 0 => array:4 [ "nombre" => "A." "apellidos" => "de Freytas" "email" => array:1 [ 0 => "defreytasoftalmo@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "M." "apellidos" => "Rengel Ruiz" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "E." "apellidos" => "España Gregori" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Oftalmología, Hospital Universitario y Politécnico La Fe, Valencia, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Radiodiagnóstico, Hospital Clínico Universitario de Valencia, Valencia, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Sección de Órbita y Oculoplastia, Hospital Universitario y Politécnico La Fe, Valencia, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Proptosis como manifestación inicial de linfoma de Burkitt con afectación orbitaria" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 449 "Ancho" => 1267 "Tamanyo" => 83680 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Right ocular globe proptosis.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Burkitt's lymphoma is a highly aggressive mature B-cell neoplasia, with overexpression of c-Myc as characteristic sign. This lymphoma has 3 clinical varieties with similar histology: the endemic form, observed in equatorial African children, typically presenting as a jawbone or orbital tumor, nearly always associated to the Epstein–Barr virus (EBV); the sporadic form, presenting as large abdominal masses usually associated to ileocecal disease,<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1,2</span></a> rarely with orbital involvement<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3,4</span></a> and related to EBV in 30–50% of cases.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1,2</span></a> The third variety is associated to immunodeficiency, generally the human immunodeficiency virus (HIV), presenting mainly with nodal and bone marrow involvement and is related to EBV in varying degrees.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1,2</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Clinic case report</span><p id="par0010" class="elsevierStylePara elsevierViewall">Female, 35, who visited the emergency department referring right ocular pain with 2 months evolution and increased intensity in the last 48<span class="elsevierStyleHsp" style=""></span>h. For a few weeks, said pain associated frontal pain which did not remit with nonsteroid anti-inflammatory drugs.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Personal history included smoking habit, denying the use of other toxic substances or known systemic diseases. Patient was in treatment with oral iron due to chronic anemia and in the past 2 months had lost 5<span class="elsevierStyleHsp" style=""></span>kg of weight without loss of appetite. Ophthalmological history included right amblyopia due to strabismus with surgery during childhood and no subsequent checkups.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The general appearance of the patient indicated poor health.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Ophthalmological examination of the right eye showed proptosis with temporal and inferior displacement of the ocular globe as well as limited ocular motility in all positions except infraduction. Periocular tissue was edematous.</p><p id="par0030" class="elsevierStylePara elsevierViewall">The left eye was normal (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The patient did not refer diplopia or diminished visual acuity, which was of hand movement in the right eye and of one in the left with myopic correction. Pupil was isochoric, with right relative afferent pupil defect. Ocular fundus exploration evidenced marked vascular tortuosity and right papillary edema (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Orbital computerized action tomography (CAT) was urgently requested, which evidenced an apparently aggressive extraconal medial lesion in the right orbit that eroded the ethmoidal lamina papyracea and occupied the ethmoidal cells, associating occupation of the frontal sinuses. There was no association of inflammatory changes in the intra- or extraconal fat (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>A and B). The differential diagnostics suggested by radiology were sarcoma or lymphoma, although this was not the typical presentation site of the latter.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">The patient was admitted for complete studies and to initiate treatment. Blood analyses identified moderate normocytic anemia (hemoglobine 9.5<span class="elsevierStyleHsp" style=""></span>g/dl), slight lymphopenia and monocitopenia.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Biopsy of the tumor was carried out endonasally and diagnosed as Burkitt's lymphoma (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>). Serologic results were positive for HIV, confirmed by means of <span class="elsevierStyleItalic">Western blot</span>.</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Discussion</span><p id="par0050" class="elsevierStylePara elsevierViewall">Burkitt's lymphoma is a highly aggressive B-cell malign neoplasia first described in 1958 as a jawbone tumor in equatorial African children.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> It is considered to be the most rapidly growing human tumor as it doubles its size in 24<span class="elsevierStyleHsp" style=""></span>h.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2,3</span></a> Multiple etiologies have been proposed for this tumor and it is the first to be associated to a virus, specifically EBV, although its role is not entirely understood.</p><p id="par0055" class="elsevierStylePara elsevierViewall">According to different clinical subtypes, Burkitt's lymphoma has different expressions. The first description was made on the endemic variety that expresses in African children aged 4–7 with frequent involvement of the jawbone and other facial bones. The sporadic form, which accounts for 1–2% of lymphomas in Weston Europe and the United States, expresses as abdominal masses that most frequently correspond to ileocecal disease, and lastly the subtypes associated to immunodeficiency which expresses more frequently in adult individuals with significant immunosuppression, to the point that this disease is considered as one of the markers of AIDS. This subtype clinically expresses as a nodal and medullar disease,<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> with orbital compromise being read,<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> as is also the case for the sporadic form.<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3,4</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Despite the low frequency of orbital involvement, it is important to take this form of presentation into account because, due to the rapid growth of the tumor, it could express with proptosis and compression of the optic nerve and quickly evolve to loss of vision that could be irreversible if not treated quickly. Accordingly, if said tumor is suspected, the patient should be submitted to a complete study and the tissue should be biopsied without delay.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conflict of interests</span><p id="par0065" class="elsevierStylePara elsevierViewall">No conflict of interest was stated by the authors.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres818610" "titulo" => "Abstract" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Case presentation" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Conclusion" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec815659" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres818609" "titulo" => "Resumen" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "abst0015" "titulo" => "Caso clínico" ] 1 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusión" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec815658" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Clinic case report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conflict of interests" ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2016-07-27" "fechaAceptado" => "2016-10-24" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec815659" "palabras" => array:5 [ 0 => "Burkitt's lymphoma" 1 => "Orbital lymphoma" 2 => "Orbital tumor" 3 => "Ocular proptosis" 4 => "Lymphoma in acquired immunodeficiency syndrome" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec815658" "palabras" => array:5 [ 0 => "Linfoma de Burkitt" 1 => "Linfoma orbitario" 2 => "Neoplasia orbitaria" 3 => "Proptosis ocular" 4 => "Linfoma en inmunodeficiencia adquirida" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Case presentation</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A 35-year-old woman without any known systemic disorder presented with a complaint of painful and rapidly increasing proptosis in her right eye. Based on the clinical, radiological, analytical and ophthalmological assessments, the diagnosis made was Burkitt's lymphoma in acquired immunodeficiency syndrome.</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conclusion</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Proptosis can be an unusual way of presenting with Burkitt's lymphoma associated with acquired immunodeficiency. This differential diagnosis is important because confirmation leads to a change in the vital prognosis of the patient.</p></span>" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Case presentation" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Conclusion" ] ] ] "es" => array:3 [ "titulo" => "Resumen" "resumen" => "<span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Caso clínico</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Una mujer de 35 años, sin antecedentes médicos conocidos, acudió a nuestro servicio de urgencias por proptosis dolorosa de rápida evolución. Los estudios de imagen mostraron una lesión que ocupaba senos frontales, etmoidales y órbita derecha. De acuerdo a la biopsia se diagnosticó de linfoma de Burkitt. La serología fue positiva para el virus de la inmunodeficiencia humana (VIH).</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Conclusión</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">La afectación orbitaria es poco frecuente en el linfoma de Burkitt asociado al VIH. Es importante tenerlo en cuenta, como diagnóstico diferencial de las proptosis, especialmente en pacientes inmunodeprimidos, por la implicación pronóstica que supone tanto a nivel sistémico como ocular.</p></span>" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "abst0015" "titulo" => "Caso clínico" ] 1 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusión" ] ] ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: de Freytas A, Rengel Ruiz M, España Gregori E. Proptosis como manifestación inicial de linfoma de Burkitt con afectación orbitaria. Arch Soc Esp Oftalmol. 2017;92:178–180.</p>" ] ] "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 449 "Ancho" => 1267 "Tamanyo" => 83680 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Right ocular globe proptosis.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1541 "Ancho" => 1591 "Tamanyo" => 172810 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Right optic nerve head hyperemia and edema.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 2312 "Ancho" => 1542 "Tamanyo" => 304486 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Cranial CAT with contrast. (A) Axial section. (B) Coronal section. Soft tissue density lesion was identified, compromising right ethmoidal cells, erosion of lamina papyracea, invasion of orbital cavity and displacement of ocular muscles (internal and superior rectus) and optic nerve.</p>" ] ] 3 => array:7 [ "identificador" => "fig0020" "etiqueta" => "Fig. 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 925 "Ancho" => 1233 "Tamanyo" => 462960 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Pathological anatomy of the lesion: proliferation of lymphoid neoplastic cells that adopt a “starry sky” pattern. 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Proptosis as initial manifestation of Burkitt's lymphoma with orbital involvement
Proptosis como manifestación inicial de linfoma de Burkitt con afectación orbitaria