Share
array:23 [ "pii" => "S2253808916000343" "issn" => "22538089" "doi" => "10.1016/j.remnie.2015.11.003" "estado" => "S300" "fechaPublicacion" => "2016-05-01" "aid" => "765" "copyright" => "Elsevier España, S.L.U. and SEMNIM" "copyrightAnyo" => "2015" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Rev Esp Med Nucl Imagen Mol. 2016;35:193-6" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 9 "PDF" => 9 ] "itemSiguiente" => array:17 [ "pii" => "S2253808916000355" "issn" => "22538089" "doi" => "10.1016/j.remnie.2015.08.003" "estado" => "S300" "fechaPublicacion" => "2016-05-01" "aid" => "740" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Rev Esp Med Nucl Imagen Mol. 2016;35:197-9" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 1 "PDF" => 1 ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Clinical note</span>" "titulo" => "<span class="elsevierStyleSup">18</span>F-FDG PET/CT in diagnosis and response evaluation in an unusual case of antisynthetase syndrome presenting as pyrexia of unknown origin" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "197" "paginaFinal" => "199" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "<span class="elsevierStyleSup">18</span>F-FDG PET/TC en el diagnóstico y evaluación de la respuesta en un caso inusual de antisintetasa síndrome que se presenta como fiebre de origen desconocido" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "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" => 1283 "Ancho" => 3252 "Tamanyo" => 387535 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Follow up PET/CT of the patient after receiving prednisolone (for 8 weeks) and cyclophosphamide (for 6 months), showed complete resolution of muscle uptake (arrow), pericardial effusion (arrow head) and significant decrease in lung infiltrates (blue arrow).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "T.K. Jain, R.K. Basher, A. Bhattacharya, B.R. Mittal, J. Shukla, M. Prakash" "autores" => array:6 [ 0 => array:2 [ "nombre" => "T.K." "apellidos" => "Jain" ] 1 => array:2 [ "nombre" => "R.K." "apellidos" => "Basher" ] 2 => array:2 [ "nombre" => "A." "apellidos" => "Bhattacharya" ] 3 => array:2 [ "nombre" => "B.R." "apellidos" => "Mittal" ] 4 => array:2 [ "nombre" => "J." "apellidos" => "Shukla" ] 5 => array:2 [ "nombre" => "M." "apellidos" => "Prakash" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2253808916000355?idApp=UINPBA00004N" "url" => "/22538089/0000003500000003/v2_201703300105/S2253808916000355/v2_201703300105/en/main.assets" ] "itemAnterior" => array:19 [ "pii" => "S2253808916000483" "issn" => "22538089" "doi" => "10.1016/j.remnie.2016.03.001" "estado" => "S300" "fechaPublicacion" => "2016-05-01" "aid" => "781" "copyright" => "Elsevier España, S.L.U. and SEMNIM" "documento" => "article" "crossmark" => 1 "subdocumento" => "sco" "cita" => "Rev Esp Med Nucl Imagen Mol. 2016;35:186-92" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 10 "formatos" => array:2 [ "HTML" => 6 "PDF" => 4 ] ] "en" => array:11 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Special collaboration</span>" "titulo" => "Usefulness of <span class="elsevierStyleSup">18</span>F-FDG PET/CT in thyroid carcinoma" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "186" "paginaFinal" => "192" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Utilidad de la <span class="elsevierStyleSup">18</span>F-FDG PET/TC en el cáncer de tiroides" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "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" => 1672 "Ancho" => 3142 "Tamanyo" => 438175 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Patient who had undergone total thyroidectomy for follicular carcinoma with a trabecular oncocytic variant with foci of clear cells. Ablative therapy with 100<span class="elsevierStyleHsp" style=""></span>mCi <span class="elsevierStyleSup">131</span>I. The patient later underwent surgery for single costal metastasis (6th left costal arch) and was treated with a new therapeutic dose of 180<span class="elsevierStyleHsp" style=""></span>mCi of <span class="elsevierStyleSup">131</span>I. Progressive increase in Tg values (28.04<span class="elsevierStyleHsp" style=""></span>ng/ml) with negative <span class="elsevierStyleSup">131</span>I whole body scan (A). A whole body <span class="elsevierStyleSup">18</span>F-FDG PET/CT scan was performed in April 2014 (B) showing hypermetabolic foci at the level of the sternum, 2nd and 10th left costal arches and left ischiopubian branch suggestive of new bone metastasis, and treatment with tyrosin kinase inhibitors (sorafenib) was initiated. Sequential studies at 6 months (October 2014) and at 14 months (June 2015) after the initiation of therapy demonstrate the persistence of the hypermetabolic foci in the axial skeleton (sternal body, 2nd and 10th left costal arches, ischiopubian branch) with a similar size and intensity of uptake. In the remainder of the study no hypermetabolic foci were observed suggesting functional stabilization of the disease. A new control <span class="elsevierStyleSup">18</span>F-FDG PET/CT study performed at 19 months after the beginning of therapy (December 2015) showed 3 foci of uptake in the nodular images in both pulmonary fields suggesting disease progression.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M.A. Muros de Fuentes, M. Mitjavila Casanovas, M. Estorch Cabrera, B. Lecumberri Santamaria, E. Navarro González" "autores" => array:5 [ 0 => array:2 [ "nombre" => "M.A." "apellidos" => "Muros de Fuentes" ] 1 => array:2 [ "nombre" => "M." "apellidos" => "Mitjavila Casanovas" ] 2 => array:2 [ "nombre" => "M." "apellidos" => "Estorch Cabrera" ] 3 => array:2 [ "nombre" => "B." "apellidos" => "Lecumberri Santamaria" ] 4 => array:2 [ "nombre" => "E." "apellidos" => "Navarro González" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S2253654X1600007X" "doi" => "10.1016/j.remn.2016.01.006" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2253654X1600007X?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2253808916000483?idApp=UINPBA00004N" "url" => "/22538089/0000003500000003/v2_201703300105/S2253808916000483/v2_201703300105/en/main.assets" ] "en" => array:19 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Clinical note</span>" "titulo" => "Bone scintigraphy as cornerstone in the diagnosis of Erdheim–Chester disease" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "193" "paginaFinal" => "196" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "F.J. García-Gómez, T. Cambil-Molina, J.J. Ríos-Martín, P.A. de la Riva-Pérez, C. Calvo-Morón, J. Castro-Montaño" "autores" => array:6 [ 0 => array:4 [ "nombre" => "F.J." "apellidos" => "García-Gómez" "email" => array:1 [ 0 => "javier191185@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" => "T." "apellidos" => "Cambil-Molina" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "J.J." "apellidos" => "Ríos-Martín" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "P.A." "apellidos" => "de la Riva-Pérez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "C." "apellidos" => "Calvo-Morón" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 5 => array:3 [ "nombre" => "J." "apellidos" => "Castro-Montaño" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Department of Nuclear Medicine, Virgen Macarena University Hospital, Seville, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Department of Pathology, Virgen Macarena University Hospital, Seville, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Papel crucial de la gammagrafía ósea en el diagnóstico de la enfermedad de Erdheim–Chester" ] ] "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" => 1252 "Ancho" => 865 "Tamanyo" => 80278 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Bone scintigraphy 120<span class="elsevierStyleHsp" style=""></span>min after intravenous injection of 888 MBq of <span class="elsevierStyleSup">99m</span>Tc-HDP, anterior and posterior views. Bone scan reveals several lesions involving the axial and appendicular skeleton (located at L1 body, left clavicle, hummers, radius and bilateral costal arches), with different rate of osteoblastic activity. A symmetrical severely intense uptake in the diaphysis and metaphysis of both femurs, metaphysis of both tibiae and heel highlights over them, and constitutes a characteristic scintigraphic pattern of ECD.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">The Erdheim–Chester disease (ECD) is an extremely non familiar entity, consisting of non-Langerhans cell histiocytosis, described at first by Jakob Erdheim and William Chester less than a century ago.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> Since then fewer than 600 cases have been reported in the literature,<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> being diagnosed more frequently over recent years probably related to the greater effort in diffusion of the disease and improvements in diagnosis techniques.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The main difficulty for diagnosis of ECD lies in the wide variety of nonspecific signs and symptoms that can occur. These signs and symptoms range from bone sclerosis, orbital infiltration with proptosis, diabetes insipidus, cardiac involvement, and pulmonary infiltration. A review of the literature tell us that bone disease is the most prevalent symptom which is usually combined with infiltration of at least one more organ system.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> This lack of specificity in symptoms and the rarity of the picture causes that there are therefore no clear-cut algorithm to guide us to obtain an optimal biopsy that confirms the diagnosis. Furthermore, optimal biopsy specimen will have important implications for the therapeutic possibilities due to new discoveries as the identification of the activating BRAF-V600 mutation.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Whole-body bone scintigraphy produces planar images of the skeleton, including anterior and posterior views of the axial and appendicular skeleton. Also additional spot views (planar images of a selected portion of the skeleton) or SPECT (tomographic image of a portion of the skeleton) are obtained as needed.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical case</span><p id="par0020" class="elsevierStylePara elsevierViewall">We present a new case recently diagnosed in the wake of the findings in bone scan. This is an 81-year-old male patient with history of diabetes insipidus who was admitted because respiratory symptoms (dyspnea and tachypnea). Severe pericardial effusion and bilateral pleural effusion were observed in the initial examination and leading to place a chest tube to drain up to 1800<span class="elsevierStyleHsp" style=""></span>ml of exudative pleural effusion with no evidence of malignant cells. Both thoracoabdominal CT and spinal MR scans (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>) were performed and revealed a moderately blastic lesion of uncertain significance in the vertebral body of L1 while pedicles were unaffected, compatible with metastatic bone disease of unknown origin. In order to skeletal mapping and according to their wide availability, a <span class="elsevierStyleSup">99m</span>Tc-HDP whole-body scan was performed (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>) demonstrating severe lesions with increased uptake of the tracer in the axial and appendicular skeleton, located at L1 body, left clavicle, hummers, radius and bilateral costal arches. These lesions presented different intensity of radiotracer uptake. Furthermore, a bilateral severely intense uptake in the diaphysis and metaphysis of both femurs, metaphysis of both tibiae and heel were observed. According to our previous experience,<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,6</span></a> we described the images as highly suggestive of ECD and indicating the need for histopathological confirmation from knee biopsy. Bone biopsy was initially reported as absence of neoplastic cells. Because of the high suspicion of ECD, the review of biopsy specimen was requested in order to specifically discard such entity (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). Foamy histiocytes positively stained for CD68 marker but S100 and CD1a negative were demonstrated after a thorough examination of samples. BRAF-V600E mutation was also confirmed. These findings allowed confirming the definitive diagnosis of ECD and discarding the neoplastic process of unknown origin which until then was the suspected diagnosis.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0025" class="elsevierStylePara elsevierViewall">ECD is a non-Langerhans cell form of histiocytosis characterized by multiorgan xanthomatous infiltration of tissues by CD68-positive, CD1a-/S100-negative foamy histiocytes. Individuals are more frequently affected in their fifth decade and there is a slight male prevalence.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> The etiology of ECD has not been elucidated. Because of its rarity and diverse presentations, it can be extremely difficult to diagnose. In most cases, patients are not properly diagnosed and managed as polypathological or cancer patients with metastasis of unknown origin. It can take years before a definitive diagnosis is made.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Biopsy is necessary to establish a definite diagnosis with the identification of stained CD68+/CD1a-/S100- foamy histiocytes. But determining the optimal place for biopsy and, subsequently, guide the pathologist to specifically discard this entity remains a challenge for physicians involved in the management of these patients. This means not only achieve correct diagnosis, but have important therapeutic and prognostic implications. Until the recent diagnosis of involvement of BRAF gene mutation, ECD patients have been treated with steroids, cladribine-based chemotherapy, and cytokine-based agents. Currently, many clinical trials based on the use of BRAF-V600E or V600K inhibitors such as vemurafenib, dabrafenib, trametinib or anakinra are being conducted.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Thus, the absence of a clear diagnosis algorithm influences in late or ineffective biopsy.</p><p id="par0040" class="elsevierStylePara elsevierViewall">In this sense, imaging techniques are a cornerstone that can help us achieving the definitive diagnosis. Because of their wide availability, thoracoabdominal CT may be the header technique, usually providing nonspecific findings as sclerotic lesions or masses mimicking metastatic disease. In this vein, 2-deoxy-2-fluoro-<span class="elsevierStyleSmallCaps">d</span>-glucose (<span class="elsevierStyleSup">18</span>F-FDG) positron emission tomography/computerized tomography (PET/CT) has shown a remarkable role in the management of this disease. <span class="elsevierStyleSup">18</span>F-FDG PET/CT allows accurate evaluation of the extent of the disease at baseline by detecting the visceral and vascular infiltration, discarding systemic involvement, locate the optimal site for biopsy, and in the assessment of response to therapy.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> Nonetheless, the use of <span class="elsevierStyleSup">18</span>F-FDG PET/CT scan is limited, owing to the high cost and low availability.</p><p id="par0045" class="elsevierStylePara elsevierViewall">On the other hand, bone scintigraphy is a non-invasive technique which is also widely available, that allows a global evaluation of skeletal abnormalities and locating silent bone involvement. Symmetric and abnormally strong labeling of the epiphysis of the long bones of the legs and sometimes the arms, is revealed by bone scintigraphy in nearly all patients,<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> becoming one of the few characteristic scintigraphic patterns. Despite the different presentations of the disease that were observed in our casuistic,<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,6</span></a> the skeletal involvement is the most widely observed reaching levels above 85% of patients in our experience (up to 96% of cases in the literature). The tibial location is additionally an accessible and relatively affordable place to take the biopsy specimen. In our opinion, whole-body bone scintigraphy because of the predominance of skeletal involvement and for being a widespread, safe, inexpensive and easy to perform technique must play a pivotal role in the diagnosis of suspected disease. Therefore, whole-body bone scan has the potential to identify ECD in an early phase of the disease and should be the technique of choice for early screening of the disease<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> when it begins to be suspected.</p><p id="par0050" class="elsevierStylePara elsevierViewall">However, this rare scintigraphic pattern may be incidentally discovered while a bone scan requested by other diagnostic suspicion is performing. In this regard, we wish to emphasize that bone biopsy is mandatory when these findings are related on a bone scan. Although bone scan is considered a highly sensitive but unspecific technique, a bilateral and symmetrical “hot knee” scintigraphic pattern has been observed and being considerate as one of the few quasi pathognomonic sign for ECD.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> We also wish to emphasize that bone biopsy is mandatory when these unusual findings are incidentally discovered on a bone scan requested by other diagnostic suspicion. The fact that there are no cancer cells in the biopsy tissue should not avoid a thorough search of disease-defining foamy cells.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusion</span><p id="par0055" class="elsevierStylePara elsevierViewall">The ECD is an extremely non-familiar disease, consisting of systemic non-Langerhans cell histiocytosis with different nonspecific clinical presentations, leading a notorious difficulty in diagnosis. Definitive diagnosis is always reached after biopsy, which also provides valuable insight into the therapeutic possibilities. Whole-body bone scintigraphy is a widely available, safe, inexpensive and easy to perform technique which shows a pathognomonic pattern of uptake for this disease, so we strongly recommend its use as screening and guiding biopsy if clinical suspicion exists. When the scintigraphic pattern is incidentally detected, biopsy of increased uptake areas (tibia preferably) is mandatory in order to discard the disease.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conflict of interest</span><p id="par0060" class="elsevierStylePara elsevierViewall">None declared.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres821702" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec818685" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres821701" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec818686" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Clinical case" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conclusion" ] 8 => array:2 [ "identificador" => "sec0025" "titulo" => "Conflict of interest" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2015-10-13" "fechaAceptado" => "2015-11-19" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec818685" "palabras" => array:3 [ 0 => "Erdheim–Chester disease" 1 => "Bone scintigraphy" 2 => "Diagnostic algorithm" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec818686" "palabras" => array:3 [ 0 => "Enfermedad de Erdheim–Chester" 1 => "Gammagrafía ósea" 2 => "Algoritmo diagnóstico" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">The Erdheim–Chester disease (ECD) is an extremely rare form of non-Langerhans cell histiocytosis. The main difficulty for its diagnosis lies in the wide variety of non-specific symptoms and signs that can occur in the disease process, leading, therefore, to there being no clear-cut algorithm as a guide for an optimal biopsy to confirm the diagnosis. An 81-year-old male with history of diabetes insipidus was admitted due to non-specific respiratory signs. Imaging techniques revealed osteoblastic lesions in the lumbar spine. Whole-body bone-scintigraphy (BS) was performed, in which lesions involving the axial and appendicular skeleton, with different rates of osteoblastic activity, were observed. This highlighted a symmetrical severely intense uptake in the knees, leading to an accurate biopsy specimen that enabled making the definitive diagnosis. BS is a widely available, safe, and inexpensive technique that shows a characteristic pattern of uptake for ECD, thus its use is highly recommended for screening and guiding biopsy if clinical suspicion exists. Furthermore, when the scintigraphy pattern is incidentally observed, biopsy of increased uptake areas (tibia preferably) is mandatory in order to rule out the disease.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La enfermedad de Erdheim–Chester es una histiocitosis no-Larngerhans extremadamente rara. La dificultad en su diagnóstico se debe a los signos y síntomas inespecíficos que presenta, que conlleva la ausencia de un claro algoritmo diagnóstico. Reportamos el caso de un varón de 81 años con diabetes insipidus en estudio por síntomas respiratorios inespecíficos. Lesiones osteoblásticas en la columna fueron referidas en técnicas radiológicas. Mediante gammagrafía ósea (GO) se observaron lesiones osteoblásticas con diferente actividad metabólica en esqueleto axial y apendicular, destacando una actividad muy elevada y simétrica en rodillas, cuya biopsia permitió el diagnóstico definitivo. La GO es una técnica disponible, segura y barata que muestra un patrón característico, por lo que recomendamos su realización como screening y guía para toma de biopsia. Ante el hallazgo incidental de dicho patrón debería realizarse biopsia.</p></span>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1529 "Ancho" => 1501 "Tamanyo" => 277594 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">(a) Thoracoabdominal CT with IV contrast, sagittal and coronal views, revealing blastic lesion in the L1 vertebral body that respects the pedicles, of uncertain significance and consistent with metastatic lesion. (b) MRI of the lumbosacral spine with and without contrast enhancement, sagittal and coronal views, showing a discrete hypointense signal in both T1- and T2-weighted sequences with slight internal heterogeneous enhancement involving the bone marrow of the vertebral body of L1. Despite its nonspecific appearance, it is suggestive of moderately osteoblastic vertebral metastasis.</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" => 1252 "Ancho" => 865 "Tamanyo" => 80278 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Bone scintigraphy 120<span class="elsevierStyleHsp" style=""></span>min after intravenous injection of 888 MBq of <span class="elsevierStyleSup">99m</span>Tc-HDP, anterior and posterior views. Bone scan reveals several lesions involving the axial and appendicular skeleton (located at L1 body, left clavicle, hummers, radius and bilateral costal arches), with different rate of osteoblastic activity. A symmetrical severely intense uptake in the diaphysis and metaphysis of both femurs, metaphysis of both tibiae and heel highlights over them, and constitutes a characteristic scintigraphic pattern of ECD.</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" => 1074 "Ancho" => 1601 "Tamanyo" => 638192 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">(a) Panoramic view of the biopsy showing widened bony trabeculae. HE, 4x. (b) Numerous foamy cells occupying intertrabecular spaces. HE, 20x. (c) Positive cytoplasmic immunostain with CD68. PAP, 40x.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Uber lipoidgranulomatose" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "W. Chester" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Virchows Arch A Pathol Anat Histopathol" "fecha" => "1930" "volumen" => "279" "paginaInicial" => "561" "paginaFinal" => "602" ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0060" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Erdheim–Chester disease: a systematic review" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:5 [ 0 => "M. Cives" 1 => "V. Simone" 2 => "F.M. Rizzo" 3 => "F. Dicuonzo" 4 => "M. Cristallo Lacalamita" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.critrevonc.2015.02.004" "Revista" => array:6 [ "tituloSerie" => "Crit Rev Oncol Hematol" "fecha" => "2015" "volumen" => "95" "paginaInicial" => "1" "paginaFinal" => "11" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/25744785" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0065" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Erdheim–Chester disease: characteristics and management" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "J. Munoz" 1 => "F. Janku" 2 => "P.R. Cohen" 3 => "R. Kurzrock" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.mayocp.2014.01.023" "Revista" => array:6 [ "tituloSerie" => "Mayo Clin Proc" "fecha" => "2014" "volumen" => "89" "paginaInicial" => "985" "paginaFinal" => "996" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24814521" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0070" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Disseminated histiocytoses biomarkers beyond BRAFV600E: frequent expression of PD-L1" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "Z. Gatalica" 1 => "N. Bilalovic" 2 => "J.P. Palazzo" 3 => "R.P. Bender" 4 => "J. Swensen" 5 => "S.Z. Millis" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.18632/oncotarget.4378" "Revista" => array:6 [ "tituloSerie" => "Oncotarget" "fecha" => "2015" "volumen" => "6" "paginaInicial" => "19819" "paginaFinal" => "19825" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26110571" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0075" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The role of 18FDG, 18FDOPA PET/CT and 99mTc bone scintigraphy imaging in Erdheim–Chester disease" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "F.J. García-Gómez" 1 => "I. Acevedo-Báñez" 2 => "R. Martínez-Castillo" 3 => "J.L. Tirado-Hospital" 4 => "J.I. Cuenca-Cuenca" 5 => "V.M. Pachón-Garrudo" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.ejrad.2015.04.022" "Revista" => array:6 [ "tituloSerie" => "Eur J Radiol" "fecha" => "2015" "volumen" => "84" "paginaInicial" => "1586" "paginaFinal" => "1592" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/25975897" "web" => "Medline" ] ] ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib0080" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Erdheim–Chester disease" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "F.J. García-Gómez" 1 => "I. Acevedo-Báñez" 2 => "E. Rivas-Infante" 3 => "I. Borrego-Dorado" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:4 [ "tituloSerie" => "Med Clin (Barc)" "fecha" => "2014" "volumen" => "143" "paginaInicial" => "e19" ] ] ] ] ] ] 6 => array:3 [ "identificador" => "bib0085" "etiqueta" => "7" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Erdheim–Chester disease" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "C. Campochiaro" 1 => "A. Tomelleri" 2 => "G. Cavalli" 3 => "A. Berti" 4 => "L. Dagna" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.ejim.2015.03.004" "Revista" => array:6 [ "tituloSerie" => "Eur J Intern Med" "fecha" => "2015" "volumen" => "26" "paginaInicial" => "223" "paginaFinal" => "229" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/25865950" "web" => "Medline" ] ] ] ] ] ] ] ] 7 => array:3 [ "identificador" => "bib0090" "etiqueta" => "8" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Erdheim–Chester disease" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J. Haroche" 1 => "L. Arnaud" 2 => "F. Cohen-Aubart" 3 => "B. Hervier" 4 => "F. Charlotte" 5 => "J.F. Emile" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s11926-014-0412-0" "Revista" => array:6 [ "tituloSerie" => "Curr Rheumatol Rep" "fecha" => "2014" "volumen" => "16" "paginaInicial" => "412" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24532298" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S0735109715060921" "estado" => "S300" "issn" => "07351097" ] ] ] ] ] ] ] 8 => array:3 [ "identificador" => "bib0095" "etiqueta" => "9" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Scintigraphic diagnosis of Erdheim–Chester disease" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "H. Balink" 1 => "M.H. Hemmelder" 2 => "W. de Graaf" 3 => "J. Grond" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1200/JCO.2010.34.0307" "Revista" => array:6 [ "tituloSerie" => "J Clin Oncol" "fecha" => "2011" "volumen" => "29" "paginaInicial" => "e470" "paginaFinal" => "e472" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21422416" "web" => "Medline" ] ] ] ] ] ] ] ] 9 => array:3 [ "identificador" => "bib0100" "etiqueta" => "10" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "High prevalence of BRAF V600E mutations in Erdheim–Chester disease but not in other non-Langerhans cell histiocytoses" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "J. Haroche" 1 => "F. Charlotte" 2 => "L. Arnaud" 3 => "V.R. Holley" 4 => "G. Cabrilo" 5 => "F. Meric-Bernstam" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1182/blood-2012-05-430140" "Revista" => array:6 [ "tituloSerie" => "Blood" "fecha" => "2012" "volumen" => "120" "paginaInicial" => "2700" "paginaFinal" => "2703" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22879539" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/22538089/0000003500000003/v2_201703300105/S2253808916000343/v2_201703300105/en/main.assets" "Apartado" => array:4 [ "identificador" => "7928" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Clinical notes" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/22538089/0000003500000003/v2_201703300105/S2253808916000343/v2_201703300105/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2253808916000343?idApp=UINPBA00004N" ]
