Case and Research Letters
Bednar Tumor (Pigmented Dermatofibrosarcoma Protuberans)
Tumor de Bednar (dermatofibrosarcoma protuberans pigmentado)
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Añón-Requena, M. Pico-Valimaña, G. Muñoz-Arias" "autores" => array:3 [ 0 => array:4 [ "nombre" => "M.J." "apellidos" => "Añón-Requena" "email" => array:1 [ 0 => "mjareq@gmail.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" => "M." "apellidos" => "Pico-Valimaña" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "G." "apellidos" => "Muñoz-Arias" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Unidad de Gestión Clínica, Intercentros de Anatomía Patológica Bahía de Cádiz, Hospital Universitario Puerto Real, Cádiz, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Sección de Dermatología, Unidad de Gestión Clínica Bloque Quirúrgico, Hospital Universitario Puerto Real, Cádiz, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Tumor de Bednar (dermatofibrosarcoma protuberans pigmentado)" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 967 "Ancho" => 1504 "Tamanyo" => 546219 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Histopathology: A, Proliferation of spindle cells and melanin-containing dendritic cells (hematoxylin-eosin, original magnification ×100). B, Melanin-containing dendritic cells and positive staining for S100 protein (S100, original magnification ×200).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Dermatofibrosarcoma protuberans (DFSP) is a dermal connective tissue tumor with low malignancy due to its slow growth and locally aggressive nature. It can be classified into several variants according to morphologic features, although there are no major differences in terms of prognosis. The pigmented variant of DFSP, also known as Bednar tumor,<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">1,2</span></a> is rare and is characterized by the presence of fibroblasts interlaced with melanin-containing dendritic cells. As with other variants of DFSP, fibrosarcomatous changes may occur; these are characterized by CD34 negativity, scarce melanin pigmentation, and increased cell proliferation and pleomorphism.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">3,4</span></a> This transformation tends to occur in recurrent DFSP and is associated with poor prognosis and an increased risk of metastasis, although the real risk is low.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The identification of the translocation t(17;22)(q22;q13) and the detection of the COL1A1-PDGFB fusion protein in DFSP led to the synthesis of tyrosine kinase inhibitors, such as imatinib as an alternative treatment for unresectable locally advanced disease or metastatic disease<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">5</span></a> and sunitinib for nonresponders to imatinib.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">6</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">We report the case of a 93-year-old man with a pigmented stain, part of which contained a firm nodular lesion of 1.5<span class="elsevierStyleHsp" style=""></span>cm, located in the left preauricular region (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). On observing that the lesion had grown, and based on a clinical suspicion of melanoma on lentigo maligna, the decision was taken to completely excise it. The histopathologic study showed focal atrophy of the epidermis, without ulceration, and a dense proliferation of monomorphic spindle cells with variable pleomorphism throughout the dermis and extending into the subcutaneous tissue (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). The spindle cells presented areas with a histiocytic/xanthomatous appearance, abundant melanic pigmentation, and isolated mitotic figures (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>A). In the immunohistochemical study, the tumor cells showed diffuse positive staining for vimentin, focal staining for CD34 and CD68, and negative staining for pan cytokeratin, actin, desmin, S100 protein, Melan-A, and HMB-45. Positive staining for S100 protein, corresponding to the dendritic cell component, was also observed in dispersed cells (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>B). Based on the above results, a diagnosis of pigmented DFSP (Bednar tumor) was established.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Pigmented DFSP was described in 1957 by Bednar<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> under the name of storiform neurofibroma, but it is currently considered to be a pigmented variant of DFSP with distinctive dendritic cells and melanin pigmentation. Its histogenesis is unclear, and it remains to be elucidated whether it is derived from an undifferentiated mesenchymal cell with the capacity to differentiate itself into a fibroblast or a histiocyte or whether, considering the presence of dendritic cells, it has a neuroectodermal origin. On occasions, onset seems to be associated with local trauma such as burns, vaccine scars, or insect bites.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">8</span></a> The differential diagnosis includes pigmented neurofibroma, cutaneous leiomyosarcoma, spindle cell squamous cell carcinoma, spindle cell melanoma, and atypical fibroxanthoma, among others. Immunohistochemistry and special techniques are essential for differentiating between these entities. The first step is to distinguish between pigmented dendritic cells (which appear brown under typical hematoxylin-eosin staining) and hemosiderin-containing macrophages. Perls stain stains the iron present in blood blue, permitting the identification of hemosiderin secondary to old bleeding. Once hemosiderin-containing macrophages have been ruled out, spindle cell proliferations with dispersed pigmented cells must be investigated by immunohistochemistry. Diffuse expression of vimentin and focal positivity for S100 protein are consistent with pigmented neurofibroma; positive staining for actin and desmin indicate a possible diagnosis of leiomyosarcoma; cytokeratin positivity suggests squamous cell carcinoma; and the expression of S100, Melan-A, and HMB-45 would indicate a possible diagnosis of melanoma. Atypical fibroxanthoma is contemplated when negative results are obtained for the above markers and the other entities in the differential diagnosis have been ruled out, although this tumor typically presents xanthomatous cells with vesicular nuclei and it may express CD10. When Bednar tumor is diagnosed, it is important to remember that these tumors are locally aggressive and invasive and tend to recur locally, but metastasis is rare and delayed. In conclusion, histopathology combined with immunohistochemistry or molecular biology is essential for the correct diagnosis and treatment of Bednar tumor.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of Interest</span><p id="par0025" 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: Añón-Requena MJ, Pico-Valimaña M, Muñoz-Arias G. Tumor de Bednar (dermatofibrosarcoma protuberans pigmentado). Actas Dermosifiliogr. 2016;107:618–620.</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" => 675 "Ancho" => 900 "Tamanyo" => 178932 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Clinical image: pigmented nodular skin lesion in the left preauricular region.</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" => 454 "Ancho" => 975 "Tamanyo" => 123973 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Histopathology: panoramic view of dermal tumor invading the subcutaneous tissue (hematoxylin-eosin, original magnification ×10).</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 967 "Ancho" => 1504 "Tamanyo" => 546219 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Histopathology: A, Proliferation of spindle cells and melanin-containing dendritic cells (hematoxylin-eosin, original magnification ×100). B, Melanin-containing dendritic cells and positive staining for S100 protein (S100, original magnification ×200).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:8 [ 0 => array:3 [ "identificador" => "bib0045" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pigmented dermatofibrosarcoma protuberans (Bednar tumor): A pathologic, ultrastructural, and immunohistochemical study" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "W.B. Dupree" 1 => "J.M. Langloss" 2 => "S.W. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 19 | 6 | 25 |
| 2024 October | 145 | 72 | 217 |
| 2024 September | 125 | 30 | 155 |
| 2024 August | 247 | 72 | 319 |
| 2024 July | 188 | 41 | 229 |
| 2024 June | 116 | 32 | 148 |
| 2024 May | 106 | 44 | 150 |
| 2024 April | 108 | 21 | 129 |
| 2024 March | 100 | 26 | 126 |
| 2024 February | 81 | 41 | 122 |
| 2024 January | 113 | 38 | 151 |
| 2023 December | 104 | 26 | 130 |
| 2023 November | 130 | 44 | 174 |
| 2023 October | 123 | 48 | 171 |
| 2023 September | 101 | 35 | 136 |
| 2023 August | 105 | 29 | 134 |
| 2023 July | 101 | 48 | 149 |
| 2023 June | 101 | 37 | 138 |
| 2023 May | 135 | 44 | 179 |
| 2023 April | 113 | 32 | 145 |
| 2023 March | 125 | 30 | 155 |
| 2023 February | 114 | 33 | 147 |
| 2023 January | 88 | 30 | 118 |
| 2022 December | 80 | 40 | 120 |
| 2022 November | 38 | 31 | 69 |
| 2022 October | 44 | 19 | 63 |
| 2022 September | 64 | 34 | 98 |
| 2022 August | 50 | 31 | 81 |
| 2022 July | 45 | 36 | 81 |
| 2022 June | 46 | 28 | 74 |
| 2022 May | 122 | 37 | 159 |
| 2022 April | 111 | 35 | 146 |
| 2022 March | 143 | 44 | 187 |
| 2022 February | 94 | 29 | 123 |
| 2022 January | 106 | 42 | 148 |
| 2021 December | 74 | 43 | 117 |
| 2021 November | 102 | 43 | 145 |
| 2021 October | 126 | 39 | 165 |
| 2021 September | 102 | 44 | 146 |
| 2021 August | 124 | 25 | 149 |
| 2021 July | 98 | 34 | 132 |
| 2021 June | 83 | 31 | 114 |
| 2021 May | 103 | 46 | 149 |
| 2021 April | 157 | 56 | 213 |
| 2021 March | 119 | 31 | 150 |
| 2021 February | 83 | 25 | 108 |
| 2021 January | 80 | 19 | 99 |
| 2020 December | 47 | 21 | 68 |
| 2020 November | 42 | 22 | 64 |
| 2020 October | 31 | 12 | 43 |
| 2020 September | 38 | 10 | 48 |
| 2020 August | 40 | 22 | 62 |
| 2020 July | 36 | 11 | 47 |
| 2020 June | 57 | 34 | 91 |
| 2020 May | 43 | 20 | 63 |
| 2020 April | 34 | 19 | 53 |
| 2020 March | 25 | 15 | 40 |
| 2020 February | 9 | 0 | 9 |
| 2020 January | 2 | 0 | 2 |
| 2019 December | 9 | 0 | 9 |
| 2019 November | 5 | 0 | 5 |
| 2019 October | 3 | 0 | 3 |
| 2019 September | 4 | 0 | 4 |
| 2019 August | 4 | 0 | 4 |
| 2019 July | 8 | 0 | 8 |
| 2019 June | 4 | 0 | 4 |
| 2019 May | 7 | 1 | 8 |
| 2019 April | 6 | 2 | 8 |
| 2019 March | 1 | 3 | 4 |
| 2019 February | 5 | 0 | 5 |
| 2019 January | 2 | 0 | 2 |
| 2018 December | 1 | 0 | 1 |
| 2018 November | 2 | 0 | 2 |
| 2018 September | 6 | 0 | 6 |
| 2018 February | 29 | 4 | 33 |
| 2018 January | 55 | 15 | 70 |
| 2017 December | 58 | 11 | 69 |
| 2017 November | 41 | 10 | 51 |
| 2017 October | 74 | 12 | 86 |
| 2017 September | 45 | 4 | 49 |
| 2017 August | 46 | 10 | 56 |
| 2017 July | 50 | 9 | 59 |
| 2017 June | 48 | 9 | 57 |
| 2017 May | 32 | 10 | 42 |
| 2017 April | 46 | 12 | 58 |
| 2017 March | 22 | 4 | 26 |
| 2017 February | 53 | 6 | 59 |
| 2017 January | 37 | 9 | 46 |
| 2016 December | 43 | 18 | 61 |
| 2016 November | 40 | 23 | 63 |
| 2016 October | 33 | 15 | 48 |
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