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Presumed solitary circumscribed retinal astrocytic proliferation
Presunta proliferación circunscrita solitaria de los astrocitos retinianos
V.M. Asensio-Sánchez
, L. Díaz-Cabanas
Corresponding author
Servicio de Oftalmología, Hospital Clínico Universitario, Valladolid, Spain
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Asensio-Sánchez, L. Díaz-Cabanas" "autores" => array:2 [ 0 => array:4 [ "nombre" => "V.M." "apellidos" => "Asensio-Sánchez" "email" => array:2 [ 0 => "victor_asensio@orangemail.es" 1 => "vmasensio@yahoo.es" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "L." "apellidos" => "Díaz-Cabanas" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Oftalmología, Hospital Clínico Universitario, Valladolid, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Presunta proliferación circunscrita solitaria de los astrocitos retinianos" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1251 "Ancho" => 1600 "Tamanyo" => 191096 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Angiography: slight hyperfluorescense in early-stage and moderate in later stage.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">The retina is a complex structure formed by neurons and glial cells (astrocytes and Müller cells). Glial cells are highly reactive to any anomalous stimuli. This reactivity is called gliosis although proliferations rarely achieve sufficient volume to simulate a tumor.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> Tumoral proliferation of retinal astrocytes is observed in benign acquired retinal astrocytoma and astrocytic hamartoma (AH) associated to tuberous sclerosis.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> Recently, a rare retinal lesion has been described involving a presumptive solitary circumscribed proliferation of retinal astrocytes (PSCPRA), with unique characteristics differentiating it from reactive gliosis and tumoral proliferation.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> A clinic case report is described which, despiste being straightforward, is relevant due to its rarity and characterization in definition pathways.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Clinic case report</span><p id="par0010" class="elsevierStylePara elsevierViewall">Male, 58, without relevant familial and personal history, who visited the practice for a routine checkup. Visual acuity was 1 in both eyes (BE). Intraocular pressure taken with applanation was 15<span class="elsevierStyleHsp" style=""></span>mm Hg in BE. Anterior segment was also normal, bilaterally. Left eye ocular fundus examination was normal, but the right eye exhibited a white-yellowish opaque lesion less than one disc diameter, with well-defined edges and located in the nasal retina inferior to the optic nerve (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The lesion did not include nutrition or drainage vessels and did not seem to exhibit intrinsic vascularization. Autofluorescence showed slight hyperautofluorescense with a small area of hypoautofluorescense in the center (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Fluorescein angiography revealed slight hyperfluorescence in the early stage and moderate and well-defined in the late stage (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Echography showed a mass without calcifications (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). Spectral domain optical coherence tomography (SD-OCT) revealed a hyper-reflective mass in snowball shape with smooth surface above the RPE and a slight optical acoustic shadow (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>). The lesion remained unchanged during a follow-up period exceeding one year.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Discussion</span><p id="par0015" class="elsevierStylePara elsevierViewall">Presumptive PSCPRA is a clinical entity recently described by Shields et al.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> in 7 patients. It is diagnosed generally in middle-aged males as a casual and asymptomatic finding in routine checkups. This tumor presents as a small, single and unilateral neoformation with well-defined edges which is white-yellowish in color, opaque, limited to the retina and remaining stable in time.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> Shields et al.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> considered that the PSCPRA acronym adequately describes the clinic characteristics of this process and, even in the absence of clear histological data, it is likely to be a proliferation of astrocytes. Recently, said authors considered that the tumor seems to originate in the external retina layers instead of the internal layers as indicated in their first description.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1,2</span></a> Schwartz and Harbour<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> presented a PSCPRA case studied with SD-OCT, suggesting that this tumor could originate in the middle layers of the retina instead of the nerve fiber layer. The patients described herein exhibited in SD-OCT a hyper-reflective mass over the RPE and Bruch's membrane. The angiographic study of the last case series described by Shields et al.,<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a> generally shows early and late hypofluorescense, matching the suspicion that it is actually an atypical fibrous metaplasia of the RPE, although angiographic behavior is variable in contrast with the relative stability of tomographic behavior. Therefore, it might not be a proliferation of astrocytes as believed, and could be a deep glial proliferation and/or a fibrous mass of the pigment epithelium.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">The differential diagnostic of a white-yellowish and well-defined retinal lesion includes AH, acquired retinal astrocytoma, retinoblastoma, retinocytoma, simple congenital RPE hamartoma, myelinized fibers, granuloma and reactive gliosis,<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1–6</span></a> although in fact the most frequent differential diagnostic is with AH. In contrast with PSCPRA, AH is usually a multiple, bilateral and translucent lesion with poorly defined edges and generally peripapillary.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">4</span></a> The majority of retinal AH are related to tuberous sclerosis or neurofibromatosis type 1. Both conditions possess dominant autosomic inheritance. Tuberous sclerosis frequently presents with cutaneous changes (sebaceous adenoma in the cheeks), cerebral astrocytomae (that cause epilepsy and mental retard) and visceral tumors in the heart (rabdomioma) and kidneys.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">7</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Neurofibromatosis type 1 is associated to changes in the skin (neurofibroma, light brown spots and freckles in the armpit or inguinal regions). Ocular expressions include plexiform neurofibromas in the eyelids and the orbits, dysplasia in the sphenoid, prominent corneal nerves, Lisch nodules in the iris and optic nerve glioma.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> In some cases, retinal AH can appear in isolation, without association to systemic diseases.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a> Acquired retinal astrocytomas tend to be progressive and could associate with exudation or exudative retinal detachment. These lesions could correspond to CNS astrocytomas.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">5</span></a> The lesion of the present patient has remained stable and unchanged, free of any exudation, and is well circumscribed and opaque, without allowing the visualization of deep vessels. All of these characteristics differentiate this lesion from other white-yellowish retinal lesions, which allowed the authors to reassure the patient about the benign prognosis.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Conflict of interests</span><p id="par0040" class="elsevierStylePara elsevierViewall">No conflict of interests was declared by the authors.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres807378" "titulo" => "Abstract" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Introduction" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Case report" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Discussion" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec805279" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres807379" "titulo" => "Resumen" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "abst0020" "titulo" => "Introducción" ] 1 => array:2 [ "identificador" => "abst0025" "titulo" => "Caso clínico" ] 2 => array:2 [ "identificador" => "abst0030" "titulo" => "Discusión" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec805280" "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" => "2016-03-14" "fechaAceptado" => "2016-05-26" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec805279" "palabras" => array:5 [ 0 => "Presumed solitary circumscribed retinal astrocytic proliferation" 1 => "Benign lesion" 2 => "Astrocytes" 3 => "Acquired retinal astrocytoma" 4 => "Astrocytic hamartoma" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec805280" "palabras" => array:5 [ 0 => "Presunta proliferación circunscrita solitaria de los astrocitos retinianos" 1 => "Lesión benigna" 2 => "Astrocitos" 3 => "Astrocitoma retiniano adquirido" 4 => "Hamartoma astrocítico" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Introduction</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Presumed solitary circumscribed retinal astrocytic proliferation (PSCRAP) is a small defined solitary lesion.</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Case report</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A 58-year-old man had an opaque yellow retinal lesion inferonasal to the optic disc. Fluorescein angiography showed mild early hyperfluorescence and late fluorescence. Ultrasound showed no calcification. Autofluorescence disclosed moderate hyperautofluorescence. Optical coherence tomography showed the mass with a snowball configuration and a smooth surface. More than one year later, the lesion was unchanged.</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Discussion</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">PSCRAP is a benign stable lesion. The main importance lies in its differentiation from well-circumscribed yellow-white lesions of the retina.</p></span>" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Introduction" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Case report" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Discussion" ] ] ] "es" => array:3 [ "titulo" => "Resumen" "resumen" => "<span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Introducción</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">La presunta proliferación circunscrita solitaria de los astrocitos retinianos (PPCSAR) es una pequeña lesión solitaria y definida.</p></span> <span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Caso clínico</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Varón de 58 años con una lesión opaca amarillenta inferonasal al disco óptico. La angiofluoresceingrafía mostró hiperfluorescencia leve en fase precoz y tardía. La ecografía no mostró calcificación. La autofluorescencia presentó autofluorescencia moderada. La tomografía de coherencia óptica mostró la masa con una configuración de bola de nieve con superficie lisa. Más de un año después, la lesión se mantuvo sin cambios.</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Discusión</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">PSCRAP es una lesión benigna estable. Es importante diferenciarla de otras lesiones retinianas blanco-amarillentas.</p></span>" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "abst0020" "titulo" => "Introducción" ] 1 => array:2 [ "identificador" => "abst0025" "titulo" => "Caso clínico" ] 2 => array:2 [ "identificador" => "abst0030" "titulo" => "Discusión" ] ] ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Asensio-Sánchez VM, Díaz-Cabanas L. Presunta proliferación circunscrita solitaria de los astrocitos retinianos. Arch Soc Esp Oftalmol. 2017;92:141–144.</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" => 1669 "Ancho" => 2333 "Tamanyo" => 407581 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Top: retinograph of both eyes. Right eye (RE): opaque tumor (preventing visualization of the vessels), white-yellowish with well-defined edges located in the nasal retina inferior to the optic nerve. Bottom: autofluorescense: RE lesion exhibits areas of slight increase and reduction of autofluorescense.</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" => 1251 "Ancho" => 1600 "Tamanyo" => 191096 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Angiography: slight hyperfluorescense in early-stage and moderate in later stage.</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" => 868 "Ancho" => 900 "Tamanyo" => 85202 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Echography: no calcification can be seen, with silent acoustic shadow at the base.</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" => 479 "Ancho" => 950 "Tamanyo" => 124180 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">SD-OCT: hyper-reflective mass with snowball configuration and smooth surface over RPE, with slight positive acoustic shadow.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:9 [ 0 => array:3 [ "identificador" => "bib0050" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Presumed solitary circumscribed retinal astrocytic proliferation" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "J.A. 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