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An uncommon cause of constitutional syndrome" "tieneTextoCompleto" => true "saludo" => "Dear Editor," "paginas" => array:1 [ 0 => array:1 [ "paginaInicial" => "239" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Eduardo L. Callejas Moraga, Iago Pinal Fernández, Luisa Roade Tato, Manel Santafé Colomina" "autores" => array:4 [ 0 => array:4 [ "nombre" => "Eduardo L." "apellidos" => "Callejas Moraga" "email" => array:1 [ 0 => "educallejasmoraga@hotmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "Iago" "apellidos" => "Pinal Fernández" ] 2 => array:2 [ "nombre" => "Luisa" "apellidos" => "Roade Tato" ] 3 => array:2 [ "nombre" => "Manel" "apellidos" => "Santafé Colomina" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Medicina Interna, Hospital Universitario Vall d’Hebron, Barcelona, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Angioma de células del litoral, una causa infrecuente de síndrome constitucional" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Described by Falk et al. in 1991,<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> littoral cell angioma consists of an infrequent splenic neoplasia, barely known and clinically asymptomatic or with non-specific symptoms. It affects both genders equally and has sometimes been related to cancer in other locations. There is a patient with constitutional syndrome attributed to the presence of this rare type of tumour, which was resolved after a splenectomy.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The patient is a 78-year old female with a history of endometrial carcinoma treated with a hysterectomy and radiotherapy, currently free from disease. She consulted for a 3-month progression of asthenia and anorexia, occasional vomiting and weight loss of 10<span class="elsevierStyleHsp" style=""></span>kg during this period, with no other accompanying symptom. Physical examination revealed a cachectic female, through an anodyne exploration using devices. Laboratory tests showed an emphasized discrete anaemia, with the remaining values for haemogram, biochemical, acute phase reactants and thyroid function within normal limits. Recurrence was ruled out for her gynaecological neoplasia through vaginal examination and ultrasound scan. A thoracoabdominal CT scan showed solid, multifocal, non-determined splenic affection, with no lymph node affection, oriented from a radiological standpoint as a probable lymphoproliferative process. A splenectomy was deemed the most convenient measure, from the diagnostic and therapeutic standpoints, and was performed with no complications. The anathomopathological spleen study showed multiple small vascular lesions (<5<span class="elsevierStyleHsp" style=""></span>mm), with lining of CD34 and CD68 positive cuboid cells, CD8 and CD31 negative cuboid cells, with no signs of malignancy, all typical for littoral cell angioma. After six months, the patient is still asymptomatic and has gained weight.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Littoral cell angioma is considered, in general, a benign splenic neoplasia, although it can sometimes be malignant.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">2,3</span></a> Clinically, it can be asymptomatic, although digestive symptoms have been described, such as nausea, vomiting and abdominal pain. Some cases may appear with accompanying toxic syndrome and/or fever of unknown origin, due to the immunological disorder which is believed to accompany this type of tumour.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1,4,5</span></a> Moreover, it has been related to Crohn's Disease, Gaucher's Disease, aplastic anaemia or myelodysplastic syndrome, among other conditions, although it is an entity in itself.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">6</span></a> However, up to one third of cases have been related to colon, kidney, pancreas and liver cancer, or other non-Hodgkin's lymphomas, testicular seminoma or gastric leiomyosarcoma, so a clinical follow-up is advisable during the first year after the diagnosis to detect potential appearance of neoplasias.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">5</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Although a CT scan-guided puncture sometimes leads to a diagnosis, a splenectomy usually confirms the diagnosis and at the same time leads to a resolution of the process. The ultrasound scan shows isoechogenic lesions in the splenic parenchyma, which reflect its solid nature, the CT scan shows masses or hypoattenuated nodes of different sizes and morphology, and the MRI shows hypointense images on T1 and T2, but none of those findings provide a diagnosis on their own.<a class="elsevierStyleCrossRefs" href="#bib0080"><span class="elsevierStyleSup">7,8</span></a> The differential diagnosis is considered for splenic vascular tumours, the most frequent splenic neoplasias, among which the angiosarcoma stands out due to its malignity. Other entities to take into consideration in the differential diagnosis of splenic lesions with such characteristics are lymphoproliferative processes, solid organ metastases, disseminating infectious diseases (tuberculosis or fungal diseases) and other inflammatory processes, such as sarcoidosis.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">In patients with constitutional syndrome and splenic lesions, like in this case, littoral cells splenic angioma is a diagnosis to take into consideration. A splenectomy in these cases provides confirmation of the diagnosis and helps to resolve the symptoms.</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: Callejas Moraga EL, Pinal Fernández I, Roade Tato L, Santafé Colomina M. Angioma de células del litoral, una causa infrecuente de síndrome constitucional. Med Clin (Barc). 2015;144:239.</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" => "Littoral cell angioma. A novel splenic vascular lesion demonstrating histiocytic differentiation" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "S. Falk" 1 => "H.J. Stutte" 2 => "G. 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Journal Information
Vol. 144. Issue 5.
Pages 239 (March 2015)
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Vol. 144. Issue 5.
Pages 239 (March 2015)
Letter to the Editor
Littoral cell angioma. An uncommon cause of constitutional syndrome
Angioma de células del litoral, una causa infrecuente de síndrome constitucional
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Eduardo L. Callejas Moraga
, Iago Pinal Fernández, Luisa Roade Tato, Manel Santafé Colomina
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Servicio de Medicina Interna, Hospital Universitario Vall d’Hebron, Barcelona, Spain
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