Cases for Diagnosis
Rapidly Growing Lesion on the Chest
Tumoración de rápido crecimiento en la zona anterior del tórax
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González-López, R.M. Ceballos-Rodríguez, E. García-Fernández" "autores" => array:3 [ 0 => array:4 [ "nombre" => "G." "apellidos" => "González-López" "email" => array:1 [ 0 => "gui.gonzalez89@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" => "R.M." "apellidos" => "Ceballos-Rodríguez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "E." "apellidos" => "García-Fernández" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario La Paz, Madrid, España" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitario La Paz, Madrid, España" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Tumoración de rápido crecimiento en la zona anterior del tórax" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 560 "Ancho" => 750 "Tamanyo" => 140531 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin-eosin, original magnification ×40.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 72-year-old man with a history of ischemic heart disease presented with an asymptomatic lesion in the midchest region. The lesion had appeared as a flat brown plaque a month earlier but had since grown and become ulcerated. The patient denied fever, asthenia, and weight loss.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">The examination revealed a brownish, indurated, ulcerated 4.5-cm plaque in the midchest region (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>) accompanied by a nonulcerated satellite lesion with a diameter of 2cm. The peripheral lymph nodes were not palpable.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Biopsy of the larger lesion showed a monomorphic, dense, diffuse infiltrate occupying the entire dermis, without extension into the hypodermis. There were no signs of epidermotropism or invasion of adjacent structures or blood vessels. The infiltrate was composed of medium-sized cells with fine chromatin and 1 or 2 nucleoli (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). The immunohistochemical study showed positive results for CD4, CD56, CD123, and TdT (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>) and negative results for other T-cell markers (CD3, CD5, CD8, and perforin). Negative results were also observed for B cell markers (CD20), myeloid markers (myeloperoxidase), and EBER.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Positron emission tomography/computed tomography results were negative except for the skin mass. A bone marrow biopsy and peripheral blood smear showed no extracutaneous involvement.</p><p id="par0025" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis?</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Blastic plasmacytoid dendritic cell neoplasm (BPDCN).</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Induction therapy was started with idarubicin on days 1 and 2 and cytarabine on days 1 to 5. This was followed by consolidation therapy with intermediate doses of cytarabine at 1 and 2 months. The treatment resulted in complete resolution of the lesions. Maintenance therapy was not administered. There have been no signs of recurrence in the 6 months following treatment initiation.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">BPDCN is an aggressive malignant hematologic disease that is included in the 2016 revision of the World Health Organization classification of myeloid neoplasms and acute leukemia.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> It is thought to originate in CD4<span class="elsevierStyleSup">+</span> and CD123<span class="elsevierStyleSup">+</span> plasmacytoid dendritic cells, although according to a recent study, it might originate in a subtype of CD56<span class="elsevierStyleSup">+</span> myeloid dendritic cells.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> It mainly affects elderly males.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Dermatologists have a key role in the diagnosis of BPDCN, as the tumor starts with skin involvement in 85% of cases.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> The presenting lesions (≤<span class="elsevierStyleHsp" style=""></span>2) are localized in 50% of cases.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Three clinical forms have been described: nodules (generally localized), ecchymotic macules, and generalized mixed lesions (nodules and macules).<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> The initial involvement is exclusively cutaneous in 64% of cases. In the remaining cases, the involvement is extracutaneous and generally affects the bone marrow, spleen, and lymph nodes.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">4,5</span></a> Peripheral blood involvement is detected by the identification of CD4<span class="elsevierStyleSup">+</span> CD56<span class="elsevierStyleSup">+</span> cells by flow cytometry.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Histologically, BPDCN is characterized by a diffuse infiltrate in the dermis and sometimes the hypodermis. The epidermis is spared. The infiltrate may be perivascular in the initial stages. Angioinvasion and angiodestruction are uncommon findings.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> Cells are medium-sized and have a blastoid morphology. Immunohistochemistry studies tend to be positive for CD4 and CD56 and for the plasmacytoid dendritic cell markers CD123, TCL-1, and CD303. They are negative for myeloid markers, such as lysozyme and myeloperoxidase, enabling differentiation between BPDCN and the main entity in the differential diagnosis: myeloid leukemia cutis. B-cell and T-cell markers are negative.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Median survival is 15 months.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Recent data have not shown any differences in survival between patients with localized and generalized BPDCN or between those with and without extracutaneous involvement at diagnosis.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> The treatment of choice is chemotherapy, although radiation therapy is an option for localized disease in elderly patients.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> Chemotherapy regimens used in acute lymphoblastic leukemia have shown the best results to date in BPDCN, with most patients showing complete response.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> Recurrence, however, is the norm and as such allogeneic hematopoietic transplantation is the only curative option for young patients without comorbidities.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of Interest</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Medical History" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Physical Examination" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Histopathology" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Additional Tests" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Diagnosis" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Clinical Course and Treatment" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Comment" ] 7 => array:2 [ "identificador" => "sec0040" "titulo" => "Conflicts of Interest" ] 8 => 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: González-López G, Ceballos-Rodríguez RM, García-Fernández E. Rapidly Growing Lesion on the Chest. Actas Dermosifiliogr. 2018;109:821–822.</p>" ] ] "multimedia" => array:3 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 563 "Ancho" => 750 "Tamanyo" => 57413 ] ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 560 "Ancho" => 750 "Tamanyo" => 140531 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin-eosin, original magnification ×40.</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" => 570 "Ancho" => 750 "Tamanyo" => 170286 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Inmunohistochemistry (CD56), original magnification ×20.</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" => "The 2016 revision to the world health organization classification of myeloid neoplasms and acute leukemia" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "D.A. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 8 | 8 | 16 |
| 2024 October | 59 | 39 | 98 |
| 2024 September | 57 | 24 | 81 |
| 2024 August | 78 | 65 | 143 |
| 2024 July | 78 | 26 | 104 |
| 2024 June | 77 | 37 | 114 |
| 2024 May | 61 | 28 | 89 |
| 2024 April | 64 | 29 | 93 |
| 2024 March | 53 | 24 | 77 |
| 2024 February | 44 | 33 | 77 |
| 2024 January | 55 | 32 | 87 |
| 2023 December | 42 | 19 | 61 |
| 2023 November | 52 | 27 | 79 |
| 2023 October | 36 | 18 | 54 |
| 2023 September | 45 | 27 | 72 |
| 2023 August | 16 | 18 | 34 |
| 2023 July | 30 | 26 | 56 |
| 2023 June | 30 | 25 | 55 |
| 2023 May | 32 | 23 | 55 |
| 2023 April | 31 | 16 | 47 |
| 2023 March | 67 | 31 | 98 |
| 2023 February | 55 | 29 | 84 |
| 2023 January | 37 | 32 | 69 |
| 2022 December | 40 | 34 | 74 |
| 2022 November | 50 | 43 | 93 |
| 2022 October | 25 | 29 | 54 |
| 2022 September | 20 | 36 | 56 |
| 2022 August | 18 | 33 | 51 |
| 2022 July | 20 | 33 | 53 |
| 2022 June | 23 | 28 | 51 |
| 2022 May | 28 | 35 | 63 |
| 2022 April | 27 | 39 | 66 |
| 2022 March | 32 | 39 | 71 |
| 2022 February | 32 | 21 | 53 |
| 2022 January | 30 | 51 | 81 |
| 2021 December | 26 | 45 | 71 |
| 2021 November | 33 | 53 | 86 |
| 2021 October | 37 | 50 | 87 |
| 2021 September | 30 | 43 | 73 |
| 2021 August | 37 | 32 | 69 |
| 2021 July | 26 | 25 | 51 |
| 2021 June | 24 | 28 | 52 |
| 2021 May | 43 | 54 | 97 |
| 2021 April | 81 | 92 | 173 |
| 2021 March | 56 | 38 | 94 |
| 2021 February | 67 | 34 | 101 |
| 2021 January | 48 | 39 | 87 |
| 2020 December | 44 | 32 | 76 |
| 2020 November | 30 | 38 | 68 |
| 2020 October | 23 | 23 | 46 |
| 2020 September | 25 | 19 | 44 |
| 2020 August | 22 | 29 | 51 |
| 2020 July | 18 | 20 | 38 |
| 2020 June | 33 | 32 | 65 |
| 2020 May | 17 | 15 | 32 |
| 2020 April | 22 | 16 | 38 |
| 2020 March | 19 | 10 | 29 |
| 2020 February | 3 | 2 | 5 |
| 2019 May | 1 | 0 | 1 |
| 2018 November | 1 | 0 | 1 |
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