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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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 19 | 6 | 25 |
| 2024 Octubre | 145 | 72 | 217 |
| 2024 Septiembre | 125 | 30 | 155 |
| 2024 Agosto | 247 | 72 | 319 |
| 2024 Julio | 188 | 41 | 229 |
| 2024 Junio | 116 | 32 | 148 |
| 2024 Mayo | 106 | 44 | 150 |
| 2024 Abril | 108 | 21 | 129 |
| 2024 Marzo | 100 | 26 | 126 |
| 2024 Febrero | 81 | 41 | 122 |
| 2024 Enero | 113 | 38 | 151 |
| 2023 Diciembre | 104 | 26 | 130 |
| 2023 Noviembre | 130 | 44 | 174 |
| 2023 Octubre | 123 | 48 | 171 |
| 2023 Septiembre | 101 | 35 | 136 |
| 2023 Agosto | 105 | 29 | 134 |
| 2023 Julio | 101 | 48 | 149 |
| 2023 Junio | 101 | 37 | 138 |
| 2023 Mayo | 135 | 44 | 179 |
| 2023 Abril | 113 | 32 | 145 |
| 2023 Marzo | 125 | 30 | 155 |
| 2023 Febrero | 114 | 33 | 147 |
| 2023 Enero | 88 | 30 | 118 |
| 2022 Diciembre | 80 | 40 | 120 |
| 2022 Noviembre | 38 | 31 | 69 |
| 2022 Octubre | 44 | 19 | 63 |
| 2022 Septiembre | 64 | 34 | 98 |
| 2022 Agosto | 50 | 31 | 81 |
| 2022 Julio | 45 | 36 | 81 |
| 2022 Junio | 46 | 28 | 74 |
| 2022 Mayo | 122 | 37 | 159 |
| 2022 Abril | 111 | 35 | 146 |
| 2022 Marzo | 143 | 44 | 187 |
| 2022 Febrero | 94 | 29 | 123 |
| 2022 Enero | 106 | 42 | 148 |
| 2021 Diciembre | 74 | 43 | 117 |
| 2021 Noviembre | 102 | 43 | 145 |
| 2021 Octubre | 126 | 39 | 165 |
| 2021 Septiembre | 102 | 44 | 146 |
| 2021 Agosto | 124 | 25 | 149 |
| 2021 Julio | 98 | 34 | 132 |
| 2021 Junio | 83 | 31 | 114 |
| 2021 Mayo | 103 | 46 | 149 |
| 2021 Abril | 157 | 56 | 213 |
| 2021 Marzo | 119 | 31 | 150 |
| 2021 Febrero | 83 | 25 | 108 |
| 2021 Enero | 80 | 19 | 99 |
| 2020 Diciembre | 47 | 21 | 68 |
| 2020 Noviembre | 42 | 22 | 64 |
| 2020 Octubre | 31 | 12 | 43 |
| 2020 Septiembre | 38 | 10 | 48 |
| 2020 Agosto | 40 | 22 | 62 |
| 2020 Julio | 36 | 11 | 47 |
| 2020 Junio | 57 | 34 | 91 |
| 2020 Mayo | 43 | 20 | 63 |
| 2020 Abril | 34 | 19 | 53 |
| 2020 Marzo | 25 | 15 | 40 |
| 2020 Febrero | 9 | 0 | 9 |
| 2020 Enero | 2 | 0 | 2 |
| 2019 Diciembre | 9 | 0 | 9 |
| 2019 Noviembre | 5 | 0 | 5 |
| 2019 Octubre | 3 | 0 | 3 |
| 2019 Septiembre | 4 | 0 | 4 |
| 2019 Agosto | 4 | 0 | 4 |
| 2019 Julio | 8 | 0 | 8 |
| 2019 Junio | 4 | 0 | 4 |
| 2019 Mayo | 7 | 1 | 8 |
| 2019 Abril | 6 | 2 | 8 |
| 2019 Marzo | 1 | 3 | 4 |
| 2019 Febrero | 5 | 0 | 5 |
| 2019 Enero | 2 | 0 | 2 |
| 2018 Diciembre | 1 | 0 | 1 |
| 2018 Noviembre | 2 | 0 | 2 |
| 2018 Septiembre | 6 | 0 | 6 |
| 2018 Febrero | 29 | 4 | 33 |
| 2018 Enero | 55 | 15 | 70 |
| 2017 Diciembre | 58 | 11 | 69 |
| 2017 Noviembre | 41 | 10 | 51 |
| 2017 Octubre | 74 | 12 | 86 |
| 2017 Septiembre | 45 | 4 | 49 |
| 2017 Agosto | 46 | 10 | 56 |
| 2017 Julio | 50 | 9 | 59 |
| 2017 Junio | 48 | 9 | 57 |
| 2017 Mayo | 32 | 10 | 42 |
| 2017 Abril | 46 | 12 | 58 |
| 2017 Marzo | 22 | 4 | 26 |
| 2017 Febrero | 53 | 6 | 59 |
| 2017 Enero | 37 | 9 | 46 |
| 2016 Diciembre | 43 | 18 | 61 |
| 2016 Noviembre | 40 | 23 | 63 |
| 2016 Octubre | 33 | 15 | 48 |
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