Case and Research Letters
First Sonographic Description of Idiopathic Cutaneous Angiosarcoma of the Head and Neck
Primera Descripción Ecográfica del Angiosarcoma Cutáneo Idiopático de Cabeza y Cuello
Pedro Vilas Boas
, Antonio Ruedas-Martínez, Ofelia Baniandrés-Rodriguez, Cristina Ciudad-Blanco
Corresponding author
Servicio de Dermatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain
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Placa eritematoviolácea, de bordes bien definidos, en la región frontotemporal. B. Proliferación neoplásica de células endoteliales de hipercromatismo nuclear y nucleomegalia que forman espacios vasculares que disecan haces de colágeno y dermis (hematoxilina-eosina 10x).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "P. Vilas Boas, A. Ruedas Martínez, O. Baniandrés Rodriguez, C. Ciudad Blanco" "autores" => array:4 [ 0 => array:2 [ "nombre" => "P." "apellidos" => "Vilas Boas" ] 1 => array:2 [ "nombre" => "A." "apellidos" => "Ruedas Martínez" ] 2 => array:2 [ "nombre" => "O." "apellidos" => "Baniandrés Rodriguez" ] 3 => array:2 [ "nombre" => "C." 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B, Color Doppler showed increased vascularity and asymmetrically distributed medium-sized vessels in the base of the nodule. C, Increased vascularity in the non-nodular area of the lesion.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">An 89-year-old woman, with a past history of systemic hypertension, aortic stenosis, and iron deficiency anemia and on treatment with hydrochlorothiazide, lisinopril, and acetylsalicylic acid, was seen for an asymptomatic lesion that had arisen a month earlier in her left frontoparietal region. She had been treated with topical corticosteroids, which were prescribed for suspected contact eczema to hair dye. Physical examination revealed an erythematous-violaceous plaque that extended down to her cheek. The surface of the lesion presented a small nodule of vascular appearance (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>, A and B).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">B-mode ultrasound (18<span class="elsevierStyleHsp" style=""></span>MHz probe, eSaote, MyLab25Gold) showed a predominantly hypoechoic nodule of 3.7<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>16.5<span class="elsevierStyleHsp" style=""></span>mm with well-defined borders, situated in the deep dermis and subcutaneous cellular tissue (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>A). The periphery of the lesion presented mixed echogenicity, in contrast to the more hypoechoic central area. In the deep region of the lesions was a hyperechoic area that may have been posterior enhancement. The epidermis appeared as a hyperechoic band. The lesion extended diffusely peripherally, and it was more difficult to identify its borders in this plane (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>, B and<span class="elsevierStyleHsp" style=""></span>C). At the lateral borders of the lesion and in the non-nodular part of the tumor, areas of mixed echogenicity predominated over the hypoechoic areas. Color Doppler showed an irregular asymmetric neovascularization at the periphery of the lesion (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>, B and<span class="elsevierStyleHsp" style=""></span>C).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Histology performed on an ultrasound-guided punch biopsy revealed a poorly defined neoplastic proliferation in the dermis, forming of anastomosing vascular spaces growing between the collagen bundles and skin appendages; the spaces were lined by endothelial cells with nuclear hyperchromatism and nucleomegaly, with frequent pleomorphic nuclei (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B). Stain for human herpesvirus 8 was negative. Based on the clinical, ultrasound, and histopathological findings, we made a diagnosis of idiopathic angiosarcoma of the head and neck.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Idiopathic angiosarcoma of the head and neck is a rare neoplasm first described by Caro and Stubenrauch in 1945. It is formed of proliferations of cells with endothelial differentiation. The lesions initially appear as erythematous plaques that rapidly progress to violaceous nodules that bleed easily. This type of angiosarcoma grows centrifugally, and can occupy large areas of the face and neck.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">1</span></a> The clinical differential diagnosis should include entities such as rosacea,<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">2,3</span></a> lymphoma, skin metastases, pyogenic granuloma, and Kaposi sarcoma. The prognosis is poor, with a 5-year survival of less than 15%.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">4,5</span></a> Surgical excision, when possible, is the treatment of choice. Chemotherapy with taxanes and palliative radiotherapy are second line treatment.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">6</span></a> Because of our patient's advanced age and the size of the lesion, it was decided to start treatment with weekly paclitaxel at a dose of 60<span class="elsevierStyleHsp" style=""></span>mg/m<span class="elsevierStyleSup">2</span>, with a poor response.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Cutaneous angiosarcoma presents histologically as a poorly defined dermal tumor with vascular tracts that infiltrate the dermis and subcutaneous cellular tissue. The endothelium can be several layers thick and contain mitotic cells. The tumor usually dissects between the collagen fibers. Ultrasound reveals hypoechoic nodules with a mixed peripheral echogenicity. The lower echogenicity of the center of the nodules may correlate with greater tumor mass and cellularity in this central region, characteristic of neoformation. The mixed peripheral echogenicity may indicate reduced cellularity in this peripheral area and dissection of the collagen bundles by tumor cells.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Ultrasound enables us to clarify the differential diagnosis with certain other conditions, such as dermatofibrosarcoma protuberans, which is characterized by lesions with distinct echogenicity and a lobulated appearance with pseudopodia, and a weak Doppler signal peripherally.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> Kaposi sarcoma is seen as a hypoechoic lesion with an increased Doppler signal within the lesion, in contrast to angiosarcoma, in which the Doppler signal is increased peripherally.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">7,8</span></a> Pyogenic granuloma presents as a well-defined, oval hypoechoic lesion in the dermis and subcutaneous cellular tissue, with low-flow central and peripheral vascularity on Doppler study.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> Lymphangiomas are poorly vascular lesions, and this is evident on ultrasound.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">8</span></a> Angiosarcoma of the breast has been reported to present hypoechoic images in the dermis and in the subcutaneous cellular tissue, with a contour showing lobules and spicules<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">8</span></a> (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>).</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">In the literature reviewed, we found no reports that describe the ultrasound characteristics of idiopathic angiosarcoma of the head and neck; the only available descriptions are of angiosarcoma of the breast.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">8</span></a> In this first description of the ultrasound findings in cutaneous angiosarcoma of the head and neck, ultrasound made guided skin biopsy possible, improving the yield and avoiding the possible complications of blind biopsy. When surgical excision is a therapeutic option, ultrasound could be an interesting tool to help define the borders of the lesion.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of Interest</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of Interest" ] 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: Boas PV, Martínez AR, Rodriguez OB, Blanco CC. Primera descripción ecográfica del angiosarcoma cutáneo idiopático de cabeza y cuello. Actas Dermosifiliogr. 2017;108:960–962.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 4000 "Ancho" => 2300 "Tamanyo" => 1230899 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Erythematous-violaceous plaque with well-defined borders in the frontotemporal region. B, Proliferation of neoplastic endothelial cells with nuclear hyperchromatism and nucleomegaly, forming vascular spaces that dissect between the collagen bundles in the dermis. Hematoxylin and eosin, original magnification,<span class="elsevierStyleHsp" style=""></span>x10.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1688 "Ancho" => 1500 "Tamanyo" => 232605 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Hypoechoic nodule with well-defined borders that are slightly more echogenic than the center of the nodule. B, Color Doppler showed increased vascularity and asymmetrically distributed medium-sized vessels in the base of the nodule. C, Increased vascularity in the non-nodular area of the lesion.</p>" ] ] 2 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Abbreviations: AS, angiosarcoma; DFSP, dermatofibrosarcoma protuberans; KS, Kaposi sarcoma; LA, lymphangioma; PG, pyogenic granuloma.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head " align="" valign="top" scope="col"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " colspan="2" align="center" valign="top" scope="col" style="border-bottom: 2px solid black">B Mode</th><th class="td" title="table-head " colspan="2" align="center" valign="top" scope="col" style="border-bottom: 2px solid black">Doppler</th></tr><tr title="table-row"><th class="td" title="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Appearance \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Echogenicity \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Site \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">DFSP \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Lobules and pseudopodia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Hypoechoic or mixed \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">+ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Periphery \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">KS \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Lobulated \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Hypoechoic \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">+ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Center of the lesion \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">AS \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Round nodule \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Hypoechoic centrally, mixed peripherally \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">+ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Periphery \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">PG \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Round nodule \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Hypoechoic \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">+ \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Center and periphery \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">LA \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Nodule \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Mixed with hypoechoic areas \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1607290.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Ultrasound Characteristics of the Main Conditions to be Included in the Differential Diagnosis.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:8 [ 0 => array:3 [ "identificador" => "bib0045" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Angiosarcoma. 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| Year/Month | Html | Total | |
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| 2024 November | 8 | 15 | 23 |
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| 2023 October | 76 | 38 | 114 |
| 2023 September | 71 | 32 | 103 |
| 2023 August | 62 | 19 | 81 |
| 2023 July | 95 | 29 | 124 |
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| 2023 May | 81 | 21 | 102 |
| 2023 April | 90 | 14 | 104 |
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| 2022 December | 73 | 31 | 104 |
| 2022 November | 49 | 38 | 87 |
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| 2022 May | 46 | 39 | 85 |
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| 2020 October | 14 | 9 | 23 |
| 2020 September | 24 | 10 | 34 |
| 2020 August | 29 | 19 | 48 |
| 2020 July | 16 | 11 | 27 |
| 2020 June | 32 | 23 | 55 |
| 2020 May | 37 | 9 | 46 |
| 2020 April | 32 | 15 | 47 |
| 2020 March | 24 | 15 | 39 |
| 2020 February | 3 | 0 | 3 |
| 2020 January | 3 | 0 | 3 |
| 2019 December | 4 | 0 | 4 |
| 2019 November | 4 | 0 | 4 |
| 2019 September | 4 | 0 | 4 |
| 2019 August | 6 | 0 | 6 |
| 2019 July | 4 | 0 | 4 |
| 2019 June | 4 | 0 | 4 |
| 2019 May | 4 | 0 | 4 |
| 2019 April | 2 | 0 | 2 |
| 2019 March | 4 | 0 | 4 |
| 2019 February | 1 | 0 | 1 |
| 2019 January | 3 | 0 | 3 |
| 2018 December | 2 | 0 | 2 |
| 2018 November | 3 | 0 | 3 |
| 2018 October | 3 | 0 | 3 |
| 2018 September | 2 | 0 | 2 |
| 2018 July | 1 | 0 | 1 |
| 2018 February | 32 | 4 | 36 |
| 2018 January | 201 | 9 | 210 |
| 2017 December | 59 | 31 | 90 |
| 2017 November | 8 | 11 | 19 |
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