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Primary retroperitoneal amyloidosis – A challenging diagnostic
Amiloidosis retroperitoneal primaria – Un desafío diagnóstico
a Department of Radiology, Hospital of Haute Saone, René Heymes Street, n. 2, 70000 Vesoul, France
b Department of Radiology, University of Medicine, Pharmacy, Science and Technology of Târgu Mureș, Gheorghe Marinescu Street, n. 38, 540139 Târgu Mureș, Romania
c Department of Radiology, Centre Hospitalier Universitaire de Besançon, Boulevard Fleming, 25030 Besançon, France
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array:4 [ "autoresLista" => "Claudia-Gabriela Moldovanu, Thibaut Zver" "autores" => array:2 [ 0 => array:4 [ "nombre" => "Claudia-Gabriela" "apellidos" => "Moldovanu" "email" => array:1 [ 0 => "moldovanu_claudia@yahoo.com" ] "referencia" => array:3 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 2 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Thibaut" "apellidos" => "Zver" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Department of Radiology, Hospital of Haute Saone, René Heymes Street, n. 2, 70000 Vesoul, France" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Department of Radiology, University of Medicine, Pharmacy, Science and Technology of Târgu Mureș, Gheorghe Marinescu Street, n. 38, 540139 Târgu Mureș, Romania" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Department of Radiology, Centre Hospitalier Universitaire de Besançon, Boulevard Fleming, 25030 Besançon, France" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Amiloidosis retroperitoneal primaria – Un desafío diagnóstico" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 979 "Ancho" => 1405 "Tamanyo" => 173111 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Axial-view computed tomography (A, unenhanced phase; B, arterial phase; C, portal phase; D, bone window) showing irregular massive thickening with calcification in the retroperitoneum (arrows).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We report the case of a 60-year-old man presenting with weight loss (15<span class="elsevierStyleHsp" style=""></span>kg in the last month), lack of energy, abdominal pain and discomfort and lymphedema of the legs. He had a previous medical history of gout, systemic arterial hypertension (diseases which had been diagnosed 10 years earlier and had been medicated and controlled), and benign prostatic hyperplasia. He had prostate biopsy and appendectomy in the past. He did not smoke cigarettes and occasionally drinks alcohol. His medical family history were unremarkable. Initial investigations revealed with leukopenia, white blood cell count, 3.37/mm<span class="elsevierStyleSup">3</span> with 55.5% neutrophils, 30/mm<span class="elsevierStyleSup">3</span> eosinophils and 28.5% lymphocytes. Other blood investigations, including creatinine, electrolytes, liver function, and random and fasting blood sugar levels, were normal. Also, tumor markers such as CA19-9, CEA and AFP were in the normal range.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Computed tomography of the abdomen revealed massive thickening of the retroperitoneum with calcifications that encased the abdominal vessels and kidneys, but without compressing them (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>) and also, pelvic lymphadenopathy.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Extensive laboratory examinations (serum and urinary protein electrophoresis) were performed, showing a monoclonal spike in the gamma region, which indicated IgG and Bence–Jones protein lambda light chains and therefore the possibility of amyloidosis was considered. Serum-free kappa and lambda chains were 36.85 and 42.90, respectively. The analysis of a biopsy of pelvic lymphadenopathy showed deposits of amorphous eosinophilic material, hyalinization and clusters of small lymphocytes. Congo red staining demonstrated amyloid deposition. Consequently, the diagnosis of lambda light chain retroperitoneal amyloidosis was confirmed by immunohistochemistry. The patient was subsequently investigated for the underlying diseases, but there was no evidence of systemic amyloidosis, plasmacytoma, or multiple myeloma. Our patient was referred to our department of haematology and subsequently started on chemotherapy with seven cycles of bortezomib 0.7<span class="elsevierStyleHsp" style=""></span>mg/m<span class="elsevierStyleSup">2</span> and neofordex 40<span class="elsevierStyleHsp" style=""></span>mg, and his condition improved with good drug tolerance. Currently, the patient is stable and the disease has stagnated.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Amyloidosis represents a group of diseases that occurs as a consequence of an extracellular deposition of amyloid protein in organs and/or tissues.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> Historically, it can be classified as primary and secondary or systemic.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">1,2</span></a> Primary amyloidosis, also known as AL amyloidosis, is a rare entity that is difficult to diagnose.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> Retroperitoneal involvement is one of the rarest forms of presentation, being a challenge, a clinical and imaging dilemma.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">2,3</span></a> Once the diagnosis of amyloidosis is suspected, it must be confirmed by biopsy on the affected organ, with histopathological assessment using the Congo red staining.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> Treatment is largely based on steroids and with autologous stem cell transplantation in a select group of non-responsive patients.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> Early diagnosis and treatment are essential in stabilizing the disease, thereby improving the outcome and the prognosis.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">1,3</span></a></p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Acknowledgement</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors of this paper like to thank Cyril Faure from the Groupe Hospitalier de la Haute-Saône, Hospital of Haute Saone, Vesoul, France, Internal Medicine Department, for his contribution to the diagnosis of this case.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflict of interest</span><p id="par0025" class="elsevierStylePara elsevierViewall">None.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Acknowledgement" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Conflict of interest" ] 2 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 979 "Ancho" => 1405 "Tamanyo" => 173111 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Axial-view computed tomography (A, unenhanced phase; B, arterial phase; C, portal phase; D, bone window) showing irregular massive thickening with calcification in the retroperitoneum (arrows).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:3 [ 0 => array:3 [ "identificador" => "bib0020" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Amyloidosis: pathogenesis and new therapeutic options" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "G. Merlini" 1 => "D.C. Seldin" 2 => "M.A. Gertz" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1200/JCO.2010322271" "Revista" => array:6 [ "tituloSerie" => "J Clin Oncol" "fecha" => "2011" "volumen" => "29" "paginaInicial" => "1924" "paginaFinal" => "1933" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21483018" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0025" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Retroperitoneal amyloidosis as the presenting manifestation of Waldenstrom's macroglobulinaemia" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "D. Franco-Palacios" 1 => "M. Tama" 2 => "S. Samaddar" 3 => "J. Yang" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1136/bcr-2013-009329" "Revista" => array:3 [ "tituloSerie" => "BMJ Case Rep" "fecha" => "2013" "volumen" => "23" ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0030" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Primary amyloidosis of the mesentery and the retroperitoneum presenting with lymphedema" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "U. Halm" 1 => "F. Berr" 2 => "A. Tannapfel" 3 => "R. Klöppel" 4 => "R. Secknus" 5 => "J. Mössner" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/j.1572-0241" "Revista" => array:6 [ "tituloSerie" => "Am J Gastroenterol" "fecha" => "1998" "volumen" => "93" "paginaInicial" => "2299" "paginaFinal" => "2300" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/9820426" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/24443824/0000004500000009/v2_202301300757/S2444382422001675/v2_202301300757/en/main.assets" "Apartado" => array:4 [ "identificador" => "75461" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Images of the month" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/24443824/0000004500000009/v2_202301300757/S2444382422001675/v2_202301300757/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2444382422001675?idApp=UINPBA00004N" ]
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