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Intrapancreatic accessory spleen with amyloidosis: Exceptional finding for a solid pancreatic lesion
Amyloidosis en bazo accesorio intrapancreático: causa excepcional de lesión sólida pancreática
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Resonancia magnética nuclear dinámica: lesión pancreática isointensa con relación al páncreas en fase arterial (flecha) y ligeramente hiperintenso en fase venosa, sin áreas de restricción de la difusión.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">B. Depósito de amiloide de patrón larvado, llenando la pulpa roja del bazo, de forma confluente, respetando los folículos y un borde de sinusoides a su alrededor. Recuadro: depósitos bajo luz polarizada que demuestran birrefringencia verde manzana.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Alba Manuel-Vázquez, Yuri Rodrigues Figueira, José Manuel Ramia" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Alba" "apellidos" => "Manuel-Vázquez" ] 1 => array:2 [ "nombre" => "Yuri" "apellidos" => "Rodrigues Figueira" ] 2 => array:2 [ "nombre" => "José Manuel" "apellidos" => "Ramia" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2387020619300956" "doi" => "10.1016/j.medcle.2019.02.009" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020619300956?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775318305372?idApp=UINPBA00004N" "url" => "/00257753/0000015200000008/v1_201904020610/S0025775318305372/v1_201904020610/es/main.assets" ] ] "itemSiguiente" => array:19 [ "pii" => "S2387020619300968" "issn" => "23870206" "doi" => "10.1016/j.medcle.2019.02.010" "estado" => "S300" "fechaPublicacion" => "2019-04-18" "aid" => "4573" "copyright" => "Elsevier España, S.L.U." 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"paginaInicial" => "e45" "paginaFinal" => "e46" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Alba Manuel-Vázquez, Yuri Rodrigues Figueira, José Manuel Ramia" "autores" => array:3 [ 0 => array:4 [ "nombre" => "Alba" "apellidos" => "Manuel-Vázquez" "email" => array:1 [ 0 => "alba_manuel_vazquez@hotmail.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" => "Yuri" "apellidos" => "Rodrigues Figueira" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "José Manuel" "apellidos" => "Ramia" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Cirugía General y Digestiva, Hospital Universitario de Guadalajara, Guadalajara, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitario de Guadalajara, Guadalajara, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Amyloidosis en bazo accesorio intrapancreático: causa excepcional de lesión sólida pancreática" ] ] "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" => 981 "Ancho" => 2333 "Tamanyo" => 416790 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Dynamic nuclear magnetic resonance: isointense pancreatic lesion associated with pancreas in the arterial phase (arrow) and slightly hyperintense in the venous phase, without areas of restricted diffusion. (B) Deposits of amyloid of latent pattern, filling the red pulp of the spleen, confluently, respecting the follicles and a sinusoid outline around it. Inset: Deposits under polarised light that show apple green birefringence.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">The accessory spleen (AS) is a congenital anomaly derived from an embryological alteration. The pancreas is its second most frequent location (10.4–17%),<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> after the splenic hilum. Amyloidosis comprises a group of diseases characterised by abnormal deposition of amyloid protein. Primary amyloidosis (AL) is the most common subtype and mainly affects the kidney, heart and liver. Spleen involvement occurs in only 15% of patients.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">In a 57-year-old male, there was an incidental finding of a 21<span class="elsevierStyleHsp" style=""></span>mm lesion in the pancreatic tail, hyperdense, well defined, with peripancreatic adenopathies in the celiac trunk and hepatic hilum in the computed tomography. Chromogranin A analytically presented 131.1<span class="elsevierStyleHsp" style=""></span>ng/ml without hormonal hypersecretion.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The study was extended with nuclear magnetic resonance, where an isointense pancreatic lesion was observed in the arterial phase, slightly hyperintense in the venous phase, without restriction in diffusion (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A) and with endoscopic ultrasonography (EUS): three hypoechoic lesions between the pancreatic tail and the splenic hilum, resulting in suspicions of adenopathies. Fine needle aspiration (FNAP) showed no conclusive data.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">In response to a 2<span class="elsevierStyleHsp" style=""></span>cm lesion in the pancreatic tail, with adenopathies and elevation of chromogranin A, and without a certain diagnosis, surgery was indicated. The presence of the described pancreatic lesion and several hepatic nodules were observed and distal splenopancreatectomy and liver biopsy were performed (postoperative morbidity: Comprehensive Complication Index 33.7, reoperation due to pancreatic fistula grade C).</p><p id="par0025" class="elsevierStylePara elsevierViewall">Histology showed the existence of a 21<span class="elsevierStyleHsp" style=""></span>mm intrapancreatic AS in the pancreatic tail with massive extracellular deposits of amyloid, amyloid in the walls of splenic sinuses and the connective tissue in the red pulp (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B), and amyloid in the arterial walls and pancreatic arterioles, the portal tracts, the hepatic sinusoids and the space of Disse. Congo red staining was seen in the smaller deposits of the arteriolar walls, with apple green birefringence under polarised light. The diagnosis of AL amyloidosis was confirmed by immunohistochemistry in the reference centre.</p><p id="par0030" class="elsevierStylePara elsevierViewall">After histological diagnosis, the patient was derived to haematology, who completed the study: kappa light chains 7.50<span class="elsevierStyleHsp" style=""></span>mg/dl (0.33–1.94), lambda light chains 0.28<span class="elsevierStyleHsp" style=""></span>mg/dl (0.57–2.63), predominance <span class="elsevierStyleItalic">κ</span> in <span class="elsevierStyleItalic">kappa</span>/<span class="elsevierStyleItalic">lambda</span> (26.79 [0.26–1.65]), serum proteinogram normal and monoclonal gammopathy of uncertain significance in bone marrow immunophenotype (91% <span class="elsevierStyleItalic">κ</span> light chain restriction). The patient continued treatment with bortezomib-cyclophosphamide-dexamethasone with good response.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The differential diagnosis of solid pancreatic lesions includes pancreatic adenocarcinoma, primitive neuroectodermal tumour, mucinous/serous cystadenoma, mucinous adenocarcinoma, intraductal papillary mucinous neoplasia, solid pseudopapillary tumour, metastasis, and, less frequently, intrapancreatic AS.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Because of its benignity and low frequency, a preoperative diagnosis of intrapancreatic AS is a challenge. There is no diagnostic algorithm in the literature and the role of EUS-FNA is not clearly defined. In our case, since there was no diagnosis of suspicion, we decided to perform surgery to confirm the nature of the lesion.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Furthermore, AS parenchyma is indistinguishable from the splenic parenchyma, and can therefore suffer the same affections.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> Up until December 2017 there have been cases of adenocarcinoma, inflammatory pseudotumour and recurrence of immune thrombocytopenic purpura<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> in intrapancreatic AS, without evidence of amyloidosis. The surgical treatment of AS is only necessary in case of association with a haematological disease or if there is a suspicion the lesion may be associated with metastasis.</p><p id="par0050" class="elsevierStylePara elsevierViewall">In routine practice, a diagnosis of amyloidosis is made by histopathological examination and histochemical stains such as Congo red, but the definition of the subtype is often a challenge. The exact identification of the protein that causes amyloidosis is of great importance because of the appearance of markedly different treatments. Immunohistochemistry can be problematic even in the most experienced laboratories due to high background staining caused by contamination of the serum and loss of epitopes by protein crosslinking after formalin fixation.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> Therefore, laser microdissection followed by mass spectrometry is considered the standard for the typing of amyloid,<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> but it is only available in specialised centres.</p><p id="par0055" class="elsevierStylePara elsevierViewall">In conclusion, the presence of an intrapancreatic AS is an infrequent diagnosis, which must be included in the differential diagnosis of pancreatic lesions to avoid unnecessary surgeries. Consideration should be given to the considerable risk of pancreatic surgery and the serious consequences of not diagnosing a potentially malignant pancreatic lesion.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Manuel-Vázquez A, Rodrigues Figueira Y, Ramia JM. Amiloidosis en bazo accesorio intrapancreático: causa excepcional de lesión sólida pancreática. Med Clin (Barc). 2019;152:e45–e46.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 981 "Ancho" => 2333 "Tamanyo" => 416790 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Dynamic nuclear magnetic resonance: isointense pancreatic lesion associated with pancreas in the arterial phase (arrow) and slightly hyperintense in the venous phase, without areas of restricted diffusion. (B) Deposits of amyloid of latent pattern, filling the red pulp of the spleen, confluently, respecting the follicles and a sinusoid outline around it. Inset: Deposits under polarised light that show apple green birefringence.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Lesions observed in accessory spleens of 311 patients" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "B. Halpert" 1 => "F. 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| 2023 March | 1 | 2 | 3 |
| 2019 August | 1 | 2 | 3 |
