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It is more frequent in women, and the average age of diagnosis is around 65 years. The metastases are locoregional towards lymph nodes and subsequently to lung in advanced stages. It has a poor prognosis, the average survival at year 5 is around 33% and most patients die within 6 months. However, there are cases reported outside this range.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1–3</span></a> Some of the treatment options are: surgery (cornerstone of treatment in most patients), radiotherapy and/or chemotherapy. Chemotherapy along with a drug (dacarbazine, doxorubicin, epirubicin or ifosfamide) or regimens in combination with anthracyclines are frequently used in patients with advanced or metastatic disease who are not candidates for surgery.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a 74-year-old woman with a history of type 2 diabetes, overweight and nodular hyperthyroidism, colonic diverticulosis and hiatus hernia. She was on daily treatment with lorazepam 1<span class="elsevierStyleHsp" style=""></span>mg/day, quetiapine 25<span class="elsevierStyleHsp" style=""></span>mg, omeprazole 20<span class="elsevierStyleHsp" style=""></span>mg, methimazole 5<span class="elsevierStyleHsp" style=""></span>mg and vildagliptin/metformin 50/1000<span class="elsevierStyleHsp" style=""></span>mg/12<span class="elsevierStyleHsp" style=""></span>h. The patient presents to the emergency department for right lateral cervical mass with orofacial paresthesias and right facial swelling, reporting weight loss of 15<span class="elsevierStyleHsp" style=""></span>kg, without any changes in bowel movement, palpitations, tremor, nervousness and fatigue during the last 3 months. In the blood test, TSH and thyroid hormones were within normal range and corrected calcium was 10.9<span class="elsevierStyleHsp" style=""></span>mg/dl. An abdominal ultrasound was performed, showing a nodule involving the right thyroid lobe, measuring 51<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>35<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>68<span class="elsevierStyleHsp" style=""></span>mm, of heterogeneous echostructure with potentially cystic hypoechoic areas with dense content, fairly-well defined edges and presence of some area of central vascularization. Left thyroid lobe measured 13<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>21<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>46<span class="elsevierStyleHsp" style=""></span>mm, slightly inhomogeneous, some tiny cystic areas, poorly defined nodules and with normal vascularization. In a subsequent cervical MRI, marked enlargement of the right thyroid lobe was observed due to a seemingly single, very heterogeneous lesion, with a 62<span class="elsevierStyleHsp" style=""></span>mm cranial–caudal diameter, a 44<span class="elsevierStyleHsp" style=""></span>mm anterior–posterior diameter and a 47<span class="elsevierStyleHsp" style=""></span>mm transverse diameter, with well-defined edges and predominantly hypointense in T1, although with some hyperintense areas. In T2 and STIR it is very heterogeneous, showing both hyper and hypointense areas. Extensive lymphadenopathy clusters are associated in both jugular chains, highlighting one adjacent to the upper pole of the right thyroid lobe, measuring 30<span class="elsevierStyleHsp" style=""></span>mm, enhanced and with clear neoplastic appearance, as well as others with the same appearance and smaller size in both supraclavicular regions and jugular chains. Behind the right jugular vein we can find 2 clusters that look different from the rest. It has a non-enhanced hypointense mid area on T1 and a non-enhanced hyperintense mid area on T2 surrounded by hyperintensity in T2 similar to the thyroid lesion. A thoracic CT did not show the disease was spread outside the thyroid gland. The thyroid immunohistochemical analysis with core needle biopsy (CNB) revealed coexpression of the keratin pool (AE1 and AE3) and vimentin and immunoexpression of endothelial markers factor VIII and CD31. There was no expression of CD34, S100 antigen, thyroid transcription factor 1 (TTF-1), thyroglobulins or cytokeratins 7 and 20.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The diagnosis was unresectable epithelioid angiosarcoma of the thyroid gland. Therefore, treatment with doxorubicin 75<span class="elsevierStyleHsp" style=""></span>mg/m<span class="elsevierStyleSup">2</span> was initiated. In the cervical–thoracic CT scan performed 3 months later, disease progression, lung metastasis and worsening of performance status were observed, resulting in exitus lethalis one month later.</p><p id="par0020" class="elsevierStylePara elsevierViewall">The natural history of thyroid angiosarcoma has been described as a longstanding goiter, with subsequent growth of a neck mass and symptoms of local compression, as reflected in the patient's history. Histopathological diagnosis is not easy (positive for CD31 and factor VIII and negative for CK). The CD31 antigen is considered the most sensitive and specific marker of endothelial differentiation, expressed in 90% of angiosarcomas. Also, thyroglobulin might play a role in the diagnosis of these tumors.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> The differential diagnosis includes pseudoangiosarcomatous carcinomas of the thyroid gland and anaplastic carcinoma.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> Today, there is still controversy if this type of tumors are endothelial tumors with differentiation or carcinoma variants, their clear distinction not being possible in some cases.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Funding</span><p id="par0025" class="elsevierStylePara elsevierViewall">The authors declare not having received any funding to carry out this study.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Funding" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: López Castro J, Villaverde Piñeiro L, Piñeiro Fernández JC. Angiosarcoma epitelioide tiroideo no alpino en mujer anciana. Med Clin (Barc). 2019;153:474.</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" => "Invasive thyroid angiosarcoma with favorable outcome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "J. Couto" 1 => "G. Martins" 2 => "R. Santos" 3 => "A.P. Matos" 4 => "J.I. 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Sung" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3346/jkms.2003.18.6.908" "Revista" => array:6 [ "tituloSerie" => "J Korean Med Sci" "fecha" => "2003" "volumen" => "18" "paginaInicial" => "908" "paginaFinal" => "913" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/14676455" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000015300000012/v1_201912120702/S2387020619304796/v1_201912120702/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000015300000012/v1_201912120702/S2387020619304796/v1_201912120702/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020619304796?idApp=UINPBA00004N" ]
Journal Information
Vol. 153. Issue 12.
Pages 474 (December 2019)
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Vol. 153. Issue 12.
Pages 474 (December 2019)
Letter to the Editor
Nonalpine epithelioid angiosarcoma of the thyroid in an old woman
Angiosarcoma epitelioide tiroideo no alpino en una mujer anciana
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